A Retrospective Review of Primary Percutaneous Endoscopic Gastrostomy and Laparoscopic Gastrostomy Tube Placement.

OBJECTIVES: The laparoscopic-assisted gastrostomy tube placement (LAP) has increasingly become the preferred method for placing gastrostomy tubes in infants and children. The goal of this retrospective review was to examine our institutional experiences with our transition from the percutaneous endoscopic gastrostomy (PEG) procedure to LAP technique. METHODS: All patients undergoing primary PEG or LAP gastrostomy at Boston Children's Hospital between January 2010 and June 2015 were identified. The primary aim was to compare complication rates within the first 6 months after tube placement; differences in total hospital procedural costs, hospital resource utilization, and postoperative gastroesophageal reflux disease were examined. RESULTS: Nine hundred and eighty-seven patients (442 PEG and 545 LAP gastrostomy tubes) were included. No differences in total complications within 6 months were seen. Patients undergoing PEG placement had more gastrostomy-related complications (PEG 30 [6.7%] vs LAP 13 [2.4%], P = 0.0007) and cellulitis (PEG 23 [5.1%] vs LAP 2 [0.4%], P = 0.03) within the first week of placement. Patients undergoing LAP procedures had more granulation tissue episodes (PEG 19 [4.4%] vs LAP 107 [19.8%], P = 0.005). No differences in emergency room visits, hospital readmissions, or postoperative gastroesophageal reflux disease were seen, although transition to a gastrojejunal tube was higher in patients undergoing LAP procedure (PEG 20 patients [4.6%] vs LAP 51 patients [9.5%], P = 0.0008). CONCLUSIONS: Total complications were similar between patients undergoing PEG versus LAP gastrostomy tube placement. Patients with the PEG procedure had more complications within the first week of placement versus patients with the LAP procedure had more granulation skin complications.

Endoscopic Esophageal Vacuum Therapy: A Novel Therapy for Esophageal Perforations in Pediatric Patients.

BACKGROUND: Esophageal perforation is a potentially life-threatening problem if not quickly diagnosed and treated appropriately. Negative-pressure wound therapy, commercially known as V.A.C. therapy, was developed in the early 1990s and is now standard of care for chronic surface wounds, ulcers, and burns. Adapting vacuum sponge therapy for use intraluminally for perforations of the esophagus was first reported in 2008. We report the first pediatric experience on a customized esophageal vacuum-assisted closure (EVAC) device for closure of esophageal perforations. AIM: To evaluate the technical feasibility, safety, and efficacy of EVAC in a pediatric population with esophageal perforations and compare efficacy to a cohort of patients who underwent stenting for esophageal perforation. METHODS: We performed an institutional review board-approved retrospective chart review on all patients who underwent EVAC for esophageal perforations (October 2013-September 2017) and who underwent externally removable stent placement for esophageal perforation (January 2010-December 2017) at our institution. Our primary aim was to evaluate technical feasibility, efficacy, and safety in the treatment of pediatric esophageal perforations. A secondary aim was to compare the efficacy of EVAC to esophageal stenting in healing esophageal perforations in our pediatric population. RESULTS: A total of 17 patients with esophageal atresia underwent therapy for esophageal perforation. Eight sponges were placed for surgical perforation and 9 were placed after endoscopic therapy perforation. The median age of patients was 24 months with the youngest patient being 3 months of age. The success rate of EVAC to seal all esophageal perforations was 88% (15/17). The success rate was similar in both subgroups: surgical anastomotic leaks at 88% (7/8) and endoscopic therapy leaks at 89% (8/9). There were no technical failures with placement. The stent group had a total of 24 patients: 19 were placed secondary to perforations from endoscopic therapy and 5 were placed secondary to surgical anastomotic perforations. The success rate of stents to seal all esophageal perforations was 63% (15/24). The success rate in the subgroups was 74% (14/19) for endoscopic therapy leaks and 20% (1/5) for surgical anastomotic leaks. In comparing success of EVAC and stent therapy, we found a statistically significant difference in favor of EVAC in healing surgical anastomotic perforations (P = 0.032). There was, however, no statistical difference in healing endoscopic therapy perforations (P = 0.360). CONCLUSIONS: EVAC is a novel, promising technique for the treatment of esophageal perforations in a pediatric population. This treatment is comparable to esophageal stenting in iatrogenic endoscopic therapy perforations and superior to stenting surgical perforations. Further prospective studies are needed to compare the effectiveness of EVAC to esophageal stenting. Improvement in device design and customization could further improve success and ease of placement.

Endoscopic Electrocautery Incisional Therapy as a Treatment for Refractory Benign Pediatric Esophageal Strictures.

BACKGROUND AND AIM: Refractory esophageal strictures are rare conditions in pediatrics, and are often due to anastomotic, congenital, or caustic strictures. Traditional treatment options include serial dilation and surgical stricture resection; endoscopic intralesional steroid injections, mitomycin C, and externally removable stents combined with dilation have had variable success rates. Although not as widely used, endoscopic electrocautery incisional therapy (EIT) has been reported as an alternative treatment for refractory strictures in a small number of adult series. The aim of the study was to evaluate the safety and efficacy of EIT in a pediatric population with refractory esophageal strictures. METHODS: A retrospective chart review was conducted on all patients who underwent EIT for esophageal strictures (May 2011-September 2017) at our tertiary-care referral center. A total of 57 patients underwent EIT. Procedural success was defined as no stricture resection, appropriate diameter for age, and fewer than 7 dilations within 24 months of first EIT session. This corresponded to the 90th percentile of the observed number of dilations in the data. All patients included in the study had at least 2-year follow-up. RESULTS: A total of 133 EIT sessions on 58 distinct anastomotic strictures were performed on 57 patients (24 girls). The youngest patient to have EIT was 3 months old and 4.8 kg. There were 36 strictures that met the criteria for refractory stricture and 22 non-refractory (NR) strictures. The median number of dilations before EIT therapy was 8 (interquartile range [IQR]: 6-10) in the refractory group and 3 (IQR: 0-3) in the NR group. In the refractory group, 61% of the patients met the criteria for treatment success. The median number of dilations within 2 years of EIT in the refractory group was 2 (IQR: 0-4). In the NR group, 100% of the patients met criteria for success. The median number of dilations within 2 years of EIT in the NR was 1 (IQR: 0-2). The overall adverse event rate was 5.3% (7/133), with 3 major (2.3%) and 4 minor events (3%). CONCLUSIONS: EIT shows promise as an adjunct treatment option for pediatric refractory esophageal strictures and may be considered before surgical resection even in severe cases. The complication rate, albeit low, is significant, and EIT should only be considered by experienced endoscopists in close consultation with surgery. Further prospective longitudinal studies are needed to validate this treatment.

Double Supercharged Jejunal Interposition for Late Salvage of Long-gap Esophageal Atresia.

BACKGROUND: A variety of surgical techniques exist to manage long-gap esophageal atresia (LGEA), including gastric pull-up (GPU), colonic interposition (CI), jejunal interposition (JI), and distraction lengthening. Salvage reconstruction for late failure of any conduit type is a complex surgical problem fraught with technical difficulty and significant risk. Jejunal interposition can be used as a salvage procedure in the management of LGEA. However, the opposing requirements of conduit length and adequate perfusion make the procedure technically challenging. Chronic comorbidities and abdominal and thoracic adhesions may further complicate these cases. METHODS: We report a technique for the management of 3 late treatment failures of LGEA using pedicled JI in conjunction with 2 additional arterial and venous anastomoses, or double supercharging. For 2 patients who presented with failed CI, pedicled JI was performed and supercharged to internal mammary vessels as well as vasculature preserved from the prior colonic flap mesentery. The third patient presented with failed GPU and underwent pedicled JI that was supercharged caudally to the gastroepiploic vessels and cranially to the left common carotid artery. RESULTS: No flaps were lost in any patients. Median operation time was 16.5 hours. Patients were monitored postoperatively in the intensive care unit for a median of 23 days, extubated after 14 days, and discharged at 41 days. Postoperatively, all patients tolerated an oral diet by discharge and continue to enjoy oral intake of all food consistencies without dysphagia or aspiration. Follow-up time spanned 2 to 4 years (average, 3.3 years). One patient required dilatations and temporary stent for stricture, and another required removal of prominent sternal wires; otherwise, no additional procedures were performed. CONCLUSIONS: Although technically difficult, double supercharged JI should be considered as a salvage operation to restore esophageal continuity after CI or GPU failure for LGEA, when there are otherwise limited reconstructive options.

Externally removable stents in the treatment of benign recalcitrant strictures and esophageal perforations in pediatric patients with esophageal atresia.

BACKGROUND: We investigated whether removable stents, such as self-expandable plastic stents (SEPSs) and fully covered self-expandable metal stents (FCSEMSs) could provide an alternative treatment for recalcitrant strictures and esophageal perforations after esophageal atresia (EA) repair. OBJECTIVE: The primary aim of our study was to evaluate technical feasibility. Secondary aims were to evaluate safety and procedural success. DESIGN: Retrospective study. SETTING: Tertiary-care referral center. PATIENTS: A total of 24 children with EA. INTERVENTIONS: Retrospective review of all children with EA who underwent dilation and esophageal stent placement from January 2010 to February 2013 at our institution. MAIN OUTCOME MEASUREMENTS: Healing of perforation and stricture resolution at 30 and 90 days. RESULTS: A total of 41 stents (SEPSs 14, FCSEMSs 27) were placed in 24 patients with EA during the study period, including 14 who had developed esophageal leaks. Procedural success of esophageal stent placement in the treatment of refractory strictures was 39% at 30 days and 26% at 90 days. The success rate was 80% for closure of esophageal perforations with stent therapy after dilation and 25% for perforations associated with surgical repair. Adverse events of stent placement included migration (21% of SEPSs and 7% of FCSEMSs), granulation tissue (37% of FCSEMSs), and deep ulcerations (22% of FCSEMSs). LIMITATIONS: Retrospective study with small sample size. CONCLUSION: SEPSs and FCSEMSs can be placed successfully in small infants and children with a history of EA repair. The stents appear to be safe and beneficial in closing esophageal perforations, especially post-dilation. However, a high stricture recurrence rate after stent removal may limit their usefulness in treating recalcitrant esophageal anastomotic strictures.

Evolution of left-sided thoracoscopic approach for long gap esophageal atresia repair.

BACKGROUND: Left-sided repair for long gap esophageal atresia (LGEA) has been described for patients with a large leftward upper pouch, no thoracic tracheoesophageal fistula (TEF) nor tracheobronchomalacia (TBM), or as salvage plan after prior failed right-sided repair. We describe our experience with left-sided MIS traction induced growth process. METHODS: We retrospectively reviewed patients who underwent Foker process for LGEA at two institutions between December 2016 and November 2021. Patient characteristics, surgical techniques, and outcomes were reviewed. RESULTS: 71 patients underwent Foker process. Of 34 MIS cases, 28 patients (82%) underwent left-sided repair (median gap length 5 cm) at median age 4 months with median 3 (range 2-8) operations and median 13.5 (IQR 11-21) days on traction until esophageal anastomosis. 9 patients (32%) underwent completely MIS approach, whereas 5 patients (18%) converted to open at first operation and 14 patients (50%) converted to open later in the traction process. Traction was internal in 68%, external in 11%, and combination in 21%. Median follow-up was 15.4 (IQR 7.5-31.7) months after anastomosis. 14% had anastomotic leak managed with antibiotics and/or esophageal vacuum therapy. Median number of esophageal dilations was 3.5 (range 0-13). 18% required stricture resection. 39% underwent Nissen fundoplication. None have needed esophageal replacement. CONCLUSIONS: For multiple reasons including the tendency of both esophageal pouches to have a leftward bias, less tracheal compression by upper pouch, and clean field of surgery for reoperative cases, we now more commonly use left-sided approach for MIS LGEA repair compared to right side, regardless of left aortic arch. LEVEL OF EVIDENCE: Level IV Treatment Study.

The Timing of Congenital Diaphragmatic Hernia Repair on Extracorporeal Membrane Oxygenation Impacts Surgical Bleeding Risk.

BACKGROUND: The optimal timing of surgical repair for infants with congenital diaphragmatic hernia (CDH) treated with extracorporeal membrane oxygenation (ECMO) support remains controversial. The risk of surgical bleeding is considered by many centers as a primary factor in determining the preferred timing of CDH repair for infants requiring ECMO support. This study compares surgical bleeding following CDH repair on ECMO in early versus delayed fashion. METHODS: A retrospective review of 146 infants who underwent CDH repair while on ECMO support from 1995 to 2021. Early repair occurred during the first 48 h after ECMO cannulation (ER) and delayed repair after 48 h (DR). Surgical bleeding was defined by the requirement of reoperative intervention for hemostasis or decompression. RESULTS: 102 infants had ER and 44 infants DR. Surgical bleeding was more frequent in the DR group (36% vs 5%, p < 0.001) with an odds ratio of 11.7 (95% CI: 3.48-39.3, p < 0.001). Blood urea nitrogen level on the day of repair was significantly elevated among those who bled (median 63 mg/dL, IQR 20-85) vs. those who did not (median 9 mg/dL, IQR 7-13) (p < 0.0001). Duration of ECMO support was shorter in the ER group (median 13 vs 18 days, p = 0.005). Survival was not statistically different between the two groups (ER 60% vs. DR 57%, p = 0.737). CONCLUSION: We demonstrate a significantly lower incidence of bleeding and shorter duration of ECMO with early CDH repair. Azotemia was a strong risk factor for surgical bleeding associated with delayed CDH repair on ECMO. LEVEL OF EVIDENCE: Level III cohort study.

Treatment of Esophageal Perforation: Endoscopic Vacuum-Assisted Closure.

Surgical repair of type C esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is complicated by an anastomotic leak in 10%-30% of cases with associated morbidity. A novel procedure in the pediatric population, endoscopic vacuum-assisted closure (EVAC), accelerates the healing of esophageal leaks by using the effects of VAC therapy, including fluid removal and stimulation of granulation tissue formation. We report 2 additional cases of chronic esophageal leak treated with EVAC in EA patients. The first is a patient with a previously repaired type C EA/TEF and left congenital diaphragmatic hernia complicated by an infected diaphragmatic hernia patch erosion into the esophagus and colon. Additionally, we discuss a second case using EVAC for early anastomotic leak following type C EA/TEF repair in a patient who was later found to have a distal congenital esophageal stricture.

Avoiding Surgery: Endoscopic Treatment of Congenital Duodenal Stenosis.

Duodenal stenosis is a rare congenital anomaly that is typically treated surgically, although endoscopic incisional therapy (EIT) and balloon dilation are minimally invasive alternatives. We present a case of a 15-month-old male with vomiting and difficulty tolerating solid food due to severe congenital duodenal stenosis. The patient underwent EIT and serial duodenal dilation to a diameter of 20 mm, which resulted in significant symptom improvement. Intralesional corticosteroid injection (ISI) was administered to help prevent the duodenal septum from restricturing. The combination of EIT, balloon dilation, and ISI was successful in treating the patient's congenital duodenal stenosis and avoided the need for surgery. However, further studies are required to confirm the efficacy of this treatment approach in this patient population. This report highlights the potential of this minimally invasive approach as an alternative to surgical intervention in the management of congenital duodenal stenosis.

Screening for Vocal Fold Movement Impairment in Children Undergoing Esophageal and Airway Surgery.

INTRODUCTION: Children undergoing cervical and/or thoracic operations are at risk for recurrent laryngeal nerve injury, resulting in vocal fold movement impairment (VFMI). Screening for VFMI is often reserved for symptomatic patients. OBJECTIVE: Identify the prevalence of VFMI in screened preoperative patients prior to an at-risk operation to evaluate the value of screening all patients at-risk for VFMI, regardless of symptoms. METHODS: A single center, retrospective review of all patients undergoing a preoperative flexible nasolaryngoscopy between 2017 and 2021, examining the presence of VFMI and associated symptoms. RESULTS: We evaluated 297 patients with a median (IQR) age of 18 (7.8, 56.3) months and a weight of 11.3 (7.8, 17.7) kilograms. Most had a history of esophageal atresia (EA, 60%), and a prior at-risk cervical or thoracic operation (73%). Overall, 72 (24%) patients presented with VFMI (51% left, 26% right, and 22% bilateral). Of patients with VFMI, 47% did not exhibit the classic symptoms (stridor, dysphonia, and aspiration) of VFMI. Dysphonia was the most prevalent classic VFMI symptom, yet only present in 18 (25%) patients. Patients presenting with a history of at-risk surgery (OR 2.3, 95%CI 1.1, 4.8, p = 0.03), presence of a tracheostomy (OR 3.1, 95%CI 1.0, 10.0, p = 0.04), or presence of a surgical feeding tube (OR 3.1, 95%CI 1.6, 6.2, p = 0.001) were more likely to present with VFMI. CONCLUSION: Routine screening for VFMI should be considered in all at-risk patients, regardless of symptoms or prior operations, particularly in those with a history of an at-risk surgery, presence of tracheostomy, or a surgical feeding tube. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:3564-3570, 2023.

Predictors of anti-reflux procedure failure in complex esophageal atresia patients.

BACKGROUND: Anti-reflux procedures (ARP) in esophageal atresia (EA) patients can be challenging and prone to failure. These challenges become more evident with increasing complexity of EA. We sought to determine predictors of ARP failure in complex EA patients. METHODS: Single-institution retrospective review of complex EA patients (e.g. long-gap EA, esophageal strictures, hiatal hernia, and reoperative ARP) who underwent an ARP from 2002 to 2019. ARP failure was defined as hiatal hernia recurrence, wrap migration/loosening, or need for reoperation. Predictors of failure were evaluated using univariate and multivariable time-to-event analysis. RESULTS: 121 patients underwent 140 ARP at a median age of 13.5 months (IQR 7, 26.5). Nissen fundoplication (89%) was the most common ARP. Mesh (bovine pericardium) reinforcement was used in 41% of the patients. Median follow-up was 3.2 years (IQR 0.9, 5.8); 44 instances of ARP failure occurred (31%), though only 20 (14%) required reoperation. Median time to failure was 8.7 months (IQR 3.2, 25). Though fewer mesh-reinforced ARP failed (21% with vs 39% without, p = 0.02), on multivariable analysis only partial fundoplication (aHR 2.22 [95% CI 1.01-4.78]) and minimally invasive repair (aHR 2.57 [95% CI 1.12-6.01]) were significant predictors of ARP failure. CONCLUSION: In our practice of complex EA patients, where ARP fail in nearly one third of cases, a Nissen fundoplication performed via laparotomy provided the lowest risk of ARP failure.

Contemporary outcomes of the Foker process and evolution of treatment algorithms for long-gap esophageal atresia.

BACKGROUND: Esophageal growth using the Foker process (FP) for long-gap esophageal atresia (LGEA) has evolved over time. METHODS: Contemporary LGEA patients treated from 2014-2020 were compared to historical controls (2005 to <2014). RESULTS: 102 contemporary LGEA patients (type A 50%, B 18%, C 32%; 36% prior anastomotic attempt; 20 with esophagostomy) underwent either primary repair (n=23), jejunal interposition (JI; n = 14), or Foker process (FP; n = 65; 49 primary [p], 16 rescue [r]). The contemporary p-FP cohort experienced significantly fewer leaks on traction (4% vs 22%), bone fractures (2% vs 22%), anastomotic leak (12% vs 37%), and Foker failure (FP→JI; 0% vs 15%), when compared to historical p-FP patients (n = 27), all p ≤ 0.01. Patients who underwent a completely (n = 11) or partially (n = 11) minimally invasive FP experienced fewer median days paralyzed (0 vs 8 vs 17) and intubated (9 vs 15 vs 25) compared to open FP patients, respectively (all p ≤ 0.03), with equivalent leak rates (18% vs 9% vs 26%, p = 0.47). At one-year post-FP, most patients (62%) are predominantly orally fed. CONCLUSION: With continued experience and technical refinements, the Foker process has evolved with improved outcomes, less morbidity and maximal esophageal preservation.

The left-sided repair: An alternative approach for difficult esophageal atresia repair.

PURPOSE: We describe a left-sided approach for long gap esophageal atresia (LGEA) repair in patients who have a large leftward upper pouch and no significant tracheomalacia, or as a salvage strategy after prior failed right-sided repairs. METHODS: Retrospective review of patients who underwent repair via traction induced growth (Foker procedure [FP]) from 2014 to 2019 was performed. Surgical technique and post-operative outcomes were evaluated. RESULTS: Of 47 LGEA patients, 18 (38%) were approached via the left side - 94% had a left aortic arch, and 22% had prior attempts at a right-sided anastomosis. More left-sided patients underwent minimally invasive repair (39% vs 7%, p = 0.007) and internal traction (50% vs 10%, p = 0.002) compared to right-sided patients. On multivariate analysis, internal traction was associated with a decreased length of paralysis (p<0.01); length of intubation and hospital stay were similar between groups. Anastomotic leak (17% vs 20%, p = 0.80) and stricture resection (6% vs 24%, p = 0.12) rates were similar. No left-sided FP patient required additional surgery for tracheomalacia, while six right-sided patients required intervention. CONCLUSION: Left-sided FP can be considered for LGEA patients with a large leftward upper pouch or as a salvage pathway after a failed right chest approach, with similar outcomes to the right-sided approach.

Evolution, lessons learned, and contemporary outcomes of esophageal replacement with jejunum for children.

BACKGROUND: The jejunal interposition is our preferred esophageal replacement route when the native esophagus cannot be reconstructed. We report the evolution of our approach and outcomes. METHODS: The study was a single-center retrospective review of children undergoing jejunal interposition for esophageal replacement. Outcomes were compared between historical (2010-2015) and contemporary cohorts (2016-2019). RESULTS: Fifty-five patients, 58% male, median age 4 years (interquartile range 2.4-8.3), with history of esophageal atresia (87%), caustic (9%) or peptic (4%) injury, underwent a jejunal interposition (historical cohort n = 14; contemporary cohort n = 41). Duration of intubation (11 vs 6 days; P = .01), intensive care unit (22 vs 13 days; P = .03), and hospital stay (50 vs 27 days; P = .004) were shorter in the contemporary cohort. Anastomotic leaks (7% vs 5%; P = .78), anastomotic stricture resection (7% vs 10%; P = .74), and need for reoperation (57% vs 46%; P = .48) were similar between cohorts. Most reoperations were elective conduit revisions. Microvascular augmentation, used in 70% of cases, was associated with 0% anastomotic leaks vs 18% without augmentation; P = .007. With median follow-up of 1.9 years (interquartile range 1.1, 3.8), 78% of patients are predominantly orally fed. Those with preoperative oral intake were more likely to achieve consistent postoperative oral intake (87.5% vs 64%; P = .04). CONCLUSION: We have made continuous improvements in our management of patients undergoing a jejunal interposition. Of these, microvascular augmentation was associated with no anastomotic leaks. Despite its complexity and potential need for conduit revision, the jejunal interposition remains our preferred esophageal replacement, given its excellent long-term functional outcomes in these complex children who have often undergone multiple procedures before the jejunal interposition.

Effect of Posterior Tracheopexy on Risk of Recurrence in Children after Recurrent Tracheo-Esophageal Fistula Repair.

BACKGROUND: A recurrent tracheo-esophageal fistula can complicate esophageal atresia and tracheo-esophageal fistula (TEF) repair in children. Therapeutic approaches and the rate of recurrence vary widely. Most reports are limited by small cohorts and short-term follow-up, and rates of re-recurrence are substantial, making it difficult to select the treatment of choice. We aimed to review our experience with the treatment of recurrent TEF using posterior tracheopexy, focusing on operative risks and long-term outcomes. STUDY DESIGN: We conducted a retrospective review of patients with esophageal atresia TEF with recurrent TEF treated at 2 institutions from 2011 to 2020. We approach recurrent TEFs surgically. Once the TEF is divided and repaired, the membranous trachea is sutured to the anterior longitudinal ligament of the spine (posterior tracheopexy) and the esophagus is rotated into the right chest (rotational esophagoplasty), separating the suture lines widely. To detect re-recurrence, patients undergo endoscopic surveillance during follow-up. RESULTS: Sixty-two patients with a recurrent TEF were surgically treated (posterior tracheopexy/rotational esophagoplasty) at a median age of 14 months. All had significant respiratory symptoms. On referral, 24 had earlier failed endoscopic and/or surgical attempts at repair. Twenty-nine required a concomitant esophageal anastomotic stricturoplasty or stricture resection. Postoperative morbidity included 3 esophageal leaks, and 1 transient vocal cord dysfunction. We have identified no recurrences, with a median follow-up of 2.5 years, and all symptoms have resolved. CONCLUSIONS: The surgical treatment of recurrent TEFs that incorporates a posterior tracheopexy and rotational esophagoplasty is highly effective for preventing re-recurrence with low perioperative morbidity.

A perioperative bivalirudin anticoagulation protocol for neonates with congenital diaphragmatic hernia on extracorporeal membrane oxygenation.

INTRODUCTION: There is little published data regarding bivalirudin anticoagulation for surgical neonates on extracorporeal membrane oxygenation (ECMO). This study described our perioperative anticoagulation protocol and evaluated the relationship of bivalirudin dose to activated partial thrombin time (aPTT) and thromboelastography reaction time (TEG-R) monitoring assays. MATERIALS AND METHODS: Neonates with congenital diaphragmatic hernia (CDH) on ECMO and single-agent bivalirudin anticoagulation at our institution from 2016 to 2018 were included. Bivalirudin infusion rates, laboratory results, transfusions, and clinical events during the initial (cannulation to repair) and postoperative (up to 60 h post-repair) periods were recorded. RESULTS: Forty-two neonates met inclusion criteria. Bivalirudin was started at 0.16 mg/kg/h and titrated in 10-20% increments to target aPTT of 70-85 s and TEG-R of 9-12 min. All patients achieved target anticoagulation levels within the first 12 h on doses ranging from 0.12-0.36 mg/kg/h. Postoperatively, bivalirudin increased to median 0.16 (range 0.08-0.40), 0.22 (0.08-0.60), and 0.39 (0.08-0.80) mg/kg/h by 6, 24, and 60 h, respectively. On multivariable regression, no significant association of aPTT (p = 0.09) or TEG-R (p = 0.22) with bivalirudin dose was seen. Hemoglobin decrease ≥2 g/dL in 24 h occurred in 39%, but there were no reoperations, deaths, or circuit changes for thrombosis. CONCLUSIONS: This standardized perioperative bivalirudin protocol achieved target anticoagulation level quickly. Postoperative bleeding was managed without significant morbidity. Consistent dose-response relationships between bivalirudin and aPTT or TEG-R were not seen, but gradually increasing doses were needed to maintain therapeutic anticoagulation.

Giant Gastroschisis with Complete Liver Herniation: A Case Report of Two Patients.

INTRODUCTION: There are no reported survivors of gastroschisis with complete liver herniation. We describe a case report of two patients, one of whom survived. CASE #1: The patient was born with gastroschisis and herniation of the entire liver. Along with silo placement, the abdominal fascia was attached to an external traction system for growth. Complete closure was achieved at 5 months. Due to pulmonary hypoplasia, high-frequency ventilation was required. The patient is doing well, on a home ventilator wean, at 20 months. CASE #2: The patient was born prematurely with gastroschisis, total liver herniation, and a defect extending to the pericardium. A silo was attached to the fascia to provide growth of the abdominal cavity. The patient developed respiratory failure, diffuse anasarca, and renal failure. She died at 38 days of life. DISCUSSION: We report the first survivor of gastroschisis with complete liver herniation, contrasting it with a death of a similar case. The associated pulmonary hypoplasia may require long-term ventilation, the inflammatory response can lead to anasarca, and renal injury can occur from acute-on-chronic compartment syndrome. CONCLUSION: External fascial traction systems can help induce growth of the abdominal wall, allowing closure of the challenging abdomen. While critical care management is complex, survival is possible.

Descending Aortopexy and Posterior Tracheopexy for Severe Tracheomalacia and Left Mainstem Bronchomalacia.

Posterior descending aortopexy can relieve posterior intrusion of the left mainstem bronchus that may limit the effectiveness of posterior tracheobronchopexy. We review outcomes of patients undergoing both descending aortopexy and posterior tracheopexy for severe tracheobronchomalacia with posterior intrusion and left mainstem compression to determine if there were resolution of clinical symptoms and bronchoscopic evidence of improvement in airway collapse. All patients who underwent both descending aortopexy and posterior tracheopexy from October 2012 to October 2016 were retrospectively reviewed. Clinical symptoms, tracheomalacia scores based on standardized dynamic airway evaluation by anatomical region, and persistent airway intrusion requiring reoperation were collected. Data were analyzed by Wald and Wilcoxon signed-rank tests. Thirty-two patients underwent descending aortopexy and posterior tracheopexy at median age of 18 months (interquartile range 6-40 months). Median follow-up was 3 months (interquartile range 1-7 months). There were statistically significant improvements in clinical symptoms postoperatively, including cough, noisy breathing, prolonged and recurrent respiratory infections, ventilator dependence, blue spells, and brief resolved unexplained events (all P < 0.001), as well as exercise intolerance (P = 0.033), transient respiratory distress requiring positive pressure (P = 0.003), and oxygen dependence (P = 0.007). Total tracheomalacia scores improved significantly (P < 0.001), with significant segmental improvements in the middle (P = 0.003) and lower (P < 0.001) trachea, and right (P = 0.011) and left (P < 0.001) mainstem bronchi. Two patients (6%) had persistent airway intrusion requiring reoperation with anterior aortopexy or tracheopexy. Descending aortopexy and posterior tracheopexy are effective in treating severe tracheobronchomalacia and left mainstem intrusion with significant improvements in clinical symptoms and degree of airway collapse on bronchoscopy.

Supercharged Jejunal Interposition: A Reliable Esophageal Replacement in Pediatric Patients.

BACKGROUND: There is no consensus for esophageal reconstruction in the pediatric population. Long defects are commonly repaired with gastric pull-up or colonic interposition; however, jejunal interposition offers several potential advantages in children. One historical concern with jejunal interposition has been the risk of flap infarction following transposition. The use of neck and intrathoracic vessels to "supercharge" the jejunum has been reported in adults. This study reports outcomes of supercharged jejunal interposition in pediatric and young adult patients with long esophageal defects. METHODS: The authors reviewed the medical records of patients who underwent supercharged jejunal interposition for esophageal reconstruction at their institution from 2013 to 2017. The authors collected data pertaining to patient characteristics, operative technique, and postoperative outcomes. RESULTS: Twenty patients, 10 female and 10 male, aged 1.4 to 23.8 years, underwent esophageal reconstruction with supercharged jejunal interposition and were followed for a median of 1.4 years. Seventeen patients had a primary diagnosis of long-gap esophageal atresia, and three required reconstruction following caustic ingestion. Eighty percent of patients had undergone prior attempts at surgical reconstruction. Postoperatively, all conduits demonstrated coordinated peristalsis, and no flap losses were noted. Major complications occurred in seven patients, stricture dilation was performed in four patients, and there was no mortality. CONCLUSIONS: Jejunal interposition with supercharging can be safely performed for management of long esophageal gaps in the pediatric setting; it is useful where the stomach or colon has been used previously or is unavailable. Long-term outcome studies are required to determine whether jejunal interposition provides a more durable and safe conduit than gastric pull-up or colonic interposition over time. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Light-emitting diode aspiration: Distinct radiographic features and approach to management.

Foreign body aspiration is a potentially life threatening problem. The successful removal of airway foreign bodies is dependent on positively identifying the object and developing a solution to its extraction prior to attempts at retrieval. Thus, pre-operative radiographic evaluation is essential in the diagnosis and management of foreign body aspiration. The current case report describes the unique challenges in the evaluation and management of an unusual foreign body within the airway. The distinctive radiographic appearance of this foreign body allows it to be easily identified pre-operatively, and this may decrease the likelihood of operative complications and patient morbidity.