Long-term Outcomes Following Thoracoscopic Division of Vascular Rings.

OBJECTIVES: We evaluate long-term symptomatic improvement in vascular ring patients who underwent thoracoscopic division at a single quaternary pediatric surgery center. METHODS: All pediatric patients who underwent vascular ring division without Kommerell's diverticulum resection between 01/2007-12/2022 were included. Surgeries were performed by pediatric general and thoracic surgeons. Patient demographic and clinical characteristics were obtained from retrospective chart review. Data on long-term symptomatic improvement were collected with structured telephone interviews. RESULTS: 60% of patients were male. Median age at operation was 24 months (IQR: 11, 60 months) with a median weight of 11.3 kg (IQR: 8.7, 19.8 kg). All patients were symptomatic preoperatively with dysphagia being the most frequent complaint (42%), followed by chronic cough (21%). Of 41 patients eligible for the long-term follow-up survey, 8 patients with a primary diagnosis of a double arch with an atretic segment in the non-dominant arch and 9 with a right dominant arch with left ligamentum arteriosum and aberrant left subclavian artery (LSCA) were contacted and consented for participation. Median interval from surgery to survey completion was 95 months (IQR 28, 135 months). Most patients had no, or only minor, symptoms related to breathing and swallowing at the time of long-term follow-up. 88% of patients experienced postoperative symptom improvement, and only one patient reported worsening of symptoms over time. CONCLUSION: Division of an atretic arch and/or ligamentum for patients with an aberrant LSCA without Kommerell's resection may be adequate to ensure long-term improvement of breathing and swallowing problems attributable to vascular rings. LEVEL OF EVIDENCE: Level IV.

American Pediatric Surgical Association Presidential Address 2021 A Surgeon's Purpose.

Through an examination of literature and art history over the past several centuries, we can better understand what it is to be a physician and meet the needs of our patients by recognizing suffering and creating hope. Literature and historical works of art are rich in the description of children and families confronting disease and through close examination of both types of media, we can better empathize and understand our patients and families when confronted by illness.

Computational fluid dynamics modeling aiding surgical planning in a toddler with Parkes Weber syndrome.

Parkes Weber syndrome is a fast-flow and slow-flow vascular anomaly with limb overgrowth that can lead to congestive heart failure and limb ischemia. Current management strategies have focused on symptom management with focal embolization. A pediatric case with early onset heart failure is reported. We discuss the use of computational fluid dynamics (CFD) modeling to guide a surgical management strategy in a toddler with an MAP2K1 mutation.

The Right Child/Right Surgeon initiative: A position statement on pediatric surgical training, sub-specialization, and continuous certification from the American Pediatric Surgical Association.

The past 50 years have witnessed profound changes in the specialty of pediatric surgery in North America. There has been a marked increase in the number of both pediatric surgical training programs and practicing pediatric general and thoracic surgeons. Despite this trend, the population of children in the United States and the birth rate have recently remained relatively flat. Some pediatric surgeons have become "super specialists", concentrating their practices in oncology or colorectal surgery. This has the potential to result in a dilution of experience for both pediatric surgical trainees and practicing pediatric surgeons, thus limiting their ability to acquire and maintain expertise, respectively. Coincident with this, there has been a relative paradigm shift in recognition that "quality of life" is based more on maintaining a creative balance in lifestyle and is not "all about work". There has been a parallel growth in the number of practicing pediatric general and thoracic surgeons in urban settings, but we have not appreciated as much growth in rural and underserved areas, where access to pediatric surgical care remains limited and fewer pediatric general and thoracic surgeons practice. This is a complex issue, as some underserved areas are economically depressed and geographically sparse, but others are just underserved with adult providers taking care of children in settings that are often under resourced for pediatric surgical care. This problem may extend beyond the boundaries of pediatric general and thoracic surgery to other specialties. As the premier association representing all pediatric surgeons in the United States, the American Pediatric Surgical Association (APSA) has concluded that the quality of pediatric surgical care will likely decline should the status quo be allowed to continue. Therefore, APSA has initiated a Right Child/Right Surgeon initiative to consider these issues and propose some potential solutions. What follows is a brief statement of intent.

Meconium Ileus.

Cystic fibrosis is one of the most common inheritable traits in Caucasians. Meconium ileus and its potential complications are the most likely reasons that these patients will need surgical care. Surgical intervention is usually needed in the neonatal period but may also be required later in life. This article discusses the various ways cystic fibrosis can affect the gastrointestinal tract. Both the operative and nonoperative management of complicated and uncomplicated meconium ileus are discussed in the neonatal period as well as long-term issues, such as distal intestinal obstructive syndrome, fibrosing colonopathy, and rectal prolapse, all of which may be seen in older children and adults.

Thoracoscopic division of vascular rings.

BACKGROUND/PURPOSE: Vascular rings are traditionally treated via an open thoracotomy. In recent years the use of thoracoscopy has increased. Herein we report our experience with thoracoscopic division of vascular rings in pediatric patients. METHODS: We reviewed all patients who underwent thoracoscopic or open division of a vascular ring at our institution between 2007 and 2015. We analyzed patient demographics, presenting symptoms, diagnostic imaging modality, ring anatomy, operative details, complications, and symptom resolution. RESULTS: Thirty-one patients underwent thoracoscopic division of a vascular ring while sixteen had open operations. Median age was 24months in the thoracoscopic group and 13months in the open group. Operative time averaged 74min (thoracoscopic) and 95min (open). There were no mortalities at 30days. There was complete symptom resolution in 71% of thoracoscopic patients and 63% of open. Patients in the thoracoscopic group had decreased ICU admissions (10% vs. 94%), chest tube use (62% vs. 100%), chylothorax (6% vs. 38%) and overall length of stay (1.7days vs. 5days). CONCLUSIONS: Thoracoscopic division of vascular rings in pediatric patients is a feasible alternative to open division and is associated with comparable rates of symptom resolution and decreased length of hospital stay and chylothorax. LEVEL OF EVIDENCE: III.

Complications in using the vertical expandable prosthetic titanium rib (VEPTR) in children.

PURPOSE: This report describes complications using the vertical expandable prosthetic titanium rib (VEPTR) for thoracic insufficiency syndrome (TIS) at a single center. METHODS: This is a prospective cohort evaluating 65 patients with rib-rib and rib-spine VEPTR devices for TIS placed between 10/2001 and 11/2014, for children with spinal or chest wall deformity. Patients were classified using the early onset scoliosis classification system (C-EOS). RESULTS: 65 patients are available for follow up. 23 congenital scoliosis, 12 neuromuscular, 14 syndromic, 2 idiopathic and 14 not classifiable by the C-EOS system including 11 chest wall reconstructions. Average age at implantation was 6.9years (range 1.3-24.8) with average follow up 6.9years (range 0.4-14.8). 22 patients had 37 complications. Those classifiable by C-EOS had complications in the normo- and hyperkyphotic groups. Implant erosion and infection were most common. The majority of complications required one additional unplanned surgery for resolution. Two complications required abandonment of a growth-friendly strategy. CONCLUSIONS: Use of VEPTR for TIS is associated with significant and frequent complications. C-EOS suggests that complications are more likely in those with normal or hyperkyphotic curves. Most complications are managed with one unplanned surgery. VEPTR is usually salvaged and abandonment of a growth-friendly strategy is unusual.

Laboratory evaluation for pediatric patients with suspected necrotizing soft tissue infections: A case-control study.

BACKGROUND/PURPOSE: Optimal outcomes for necrotizing soft tissue infections (NSTI) depend on rapid diagnosis and management. The Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score is a validated diagnostic tool for adult NSTI, but its value for children remains unknown. We hypothesized that modification of the LRINEC score may increase its diagnostic accuracy for pediatric NSTI. METHODS: We performed a case-control study of pediatric patients (age <18) with NSTI (cases) and patients with severe soft tissue infections prompting surgical consultation (controls). The LRINEC score was calculated for cases and controls and compared to a modified, pediatric LRINEC (P-LRINEC) score. Diagnostic accuracy was analyzed through receiver operating characteristic (ROC) curves. RESULTS: From 2010 to 2014, 20 cases and 20 controls were identified at two children's hospitals. Median LRINEC score was 3.5 (1-8) for cases and 2 (1-7) for controls (p=0.03). The P-LRINEC was comprised of serum CRP >20 (sensitivity=95% (95%CI 79-100%)) and serum sodium <135 (specificity=95% (95%CI 82-100%)). Area under ROC curves was 0.70 (95%CI 0.54-0.87) for the LRINEC score and 0.84 (95%CI 0.72-0.96) for the P-LRINEC score (p=0.06). CONCLUSION: The P-LRINEC is a simplified version of the LRINEC score utilizing only CRP and sodium and may provide superior accuracy in predicting pediatric NSTI.

Understanding the Operative Experience of the Practicing Pediatric Surgeon: Implications for Training and Maintaining Competency.

IMPORTANCE: The number of practicing pediatric surgeons has increased rapidly in the past 4 decades, without a significant increase in the incidence of rare diseases specific to the field. Maintenance of competency in the index procedures for these rare diseases is essential to the future of the profession. OBJECTIVE: To describe the demographic characteristics and operative experiences of practicing pediatric surgeons using Pediatric Surgery Board recertification case log data. DESIGN, SETTING, AND PARTICIPANTS: We performed a retrospective review of 5 years of pediatric surgery certification renewal applications submitted to the Pediatric Surgery Board between 2009 and 2013. A surgeon's location was defined by population as urban, large rural, small rural, or isolated. Case log data were examined to determine case volume by category and type of procedures. Surgeons were categorized according to recertification at 10, 20, or 30 years. MAIN OUTCOME AND MEASURE: Number of index cases during the preceding year. RESULTS: Of 308 recertifying pediatric surgeons, 249 (80.8%) were men, and 143 (46.4%) were 46 to 55 years of age. Most of the pediatric surgeons (304 of 308 [98.7%]) practiced in urban areas (ie, with a population >50 000 people). All recertifying applicants were clinically active. An appendectomy was the most commonly performed procedure (with a mean [SD] number of 49.3 [35.0] procedures per year), nonoperative trauma management came in second (with 20.0 [33.0] procedures per year), and inguinal hernia repair for children younger than 6 months of age came in third (with 14.7 [13.8] procedures per year). In 6 of 10 "rare" pediatric surgery cases, the mean number of procedures was less than 2. Of 308 surgeons, 193 (62.7%) had performed a neuroblastoma resection, 170 (55.2%) a kidney tumor resection, and 123 (39.9%) an operation to treat biliary atresia or choledochal cyst in the preceding year. Laparoscopy was more frequently performed in the 10-year recertification group for Nissen fundoplication, appendectomy, splenectomy, gastrostomy/jejunostomy, orchidopexy, and cholecystectomy (P < .05) but not lung resection (P = .70). It was more frequently used by surgeons recertifying in the 10-year group (used in 11 375 of 14 456 procedures [78.7%]) than by surgeons recertifying in the 20-year (used in 6214 of 8712 procedures [71.3%]) or 30-year group (used in 2022 of 3805 procedures [53.1%]). CONCLUSIONS AND RELEVANCE: Practicing pediatric surgeons receive limited exposure to index cases after training. With regard to maintaining competency in an era in which health care outcomes have become increasingly important, these results are concerning.

Vesicoureteral reflux and febrile urinary tract infections in anorectal malformations: a retrospective review.

BACKGROUND: Multiple studies document a correlation between anorectal malformations (ARMs) and vesicoureteral reflux (VUR), development of urinary tract infections (UTIs), and subsequent renal disease. We aimed to determine which patient characteristics are associated with VUR and UTI in this population. METHODS: A retrospective review of ARM patients at a free-standing children's hospital from January 1996 to December 2011 was performed. Logistic regression was used to investigate the associations between VUR and UTI and ARM classification and co-morbid diagnoses. RESULTS: Of 190 patients, 41 (31%) received a diagnosis of VUR. Thirty-one of the 190 patients had at least one febrile UTI (16%). Of these, only 16 (51%) had a diagnosis of VUR. On multivariable logistic regression, the only patient variable associated with VUR was having an ectopic kidney (p=0.026). Similarly, the presence of GU malformations was the closest variable associated with developing a UTI (p=0.073). CONCLUSIONS: In ARM patients, VUR as well as UTIs are associated with the presence of GU malformations. Thus, voiding cystourethrogram (VCUG) testing should be pursued when there are other caudal and GU abnormalities, regardless of fistula location. Antibiotic prophylaxis for UTI should be considered in children with ARM and any GU malformation, not only VUR.

Isolated appendiceal typhlitis masquerading as perforated appendicitis in the setting of acute lymphoblastic leukemia.

Abdominal pain is common during chemotherapy for childhood leukemia. Clinically differentiating typhlitis from appendicitis can be difficult. We present an 8-year-old boy with abdominal pain in the setting of acute lymphoblastic leukemia and neutropenia. Following appendectomy for presumed appendicitis, pathology revealed appendiceal typhlitis. Diagnostic and treatment considerations are discussed.

Standardization of operative equipment reduces cost.

BACKGROUND: We hypothesize that standardizing operative equipment, and reducing variability can safely achieve cost reduction. METHODS: We retrospectively measured supply costs, operative time, intra-operative complications, and length of stay in a cohort of 145 patients at a children's hospital who underwent a laparoscopic appendectomy. A standardized preference card for laparoscopic appendectomy was developed and implemented. Data were prospectively collected on 101 consecutive patients and compared to the retrospective cohort using multiple linear regression. A survey assessing the perception of surgeons, nurses and scrub technologists of the impact of standardization on patient safety, patient care, OR efficiency, and cost was conducted. Wilcoxon rank sum test was used to evaluate associations between clinical role and years of experience with the total level of agreement on the survey. RESULTS: A 20% average reduction was achieved in supply cost per case, with no significant change in operative time (p=0.14), total time in OR (p=0.15), or length of stay (p=0.60). No intra-operative complications were identified in either group. Survey participants agreed that standardization improves cost and safety. Nurses tended to have greater agreement that standardization improved efficiency and patient care compared to other roles (p=0.06). CONCLUSIONS: Standardization of operative equipment can result in a significant cost reduction without impacting quality or delivery of care. Based on average case number per year, a total annual cost savings of >$41,000 could be realized. Survey participants agree that standardization improves cost and patient safety, yet perceptions regarding the impact on efficiency and patient care varied by occupation.

Anesthesia in a 12 year old boy with somatic overgrowth secondary to pericentric inversion of chromosome 12.

The management of a splenectomy in a boy with an unusual form of somatic overgrowth is presented. Except for a moderately difficult airway, no unusual reactions to anesthesia and surgery were encountered. Possible anesthetic implications of different somatic overgrowth syndromes in children are presented.

Intradiaphragmatic pulmonary sequestration: advantages of the thoracoscopic approach.

Pulmonary sequestrations are accessory foregut lesions that are most commonly located within the thorax and occasionally in the abdominal cavity. Sequestrations arising within the diaphragm are exceedingly rare. We describe 2 patients found to have left peridiaphragmatic lesions on prenatal ultrasound and postnatal computed tomography. In the first patient, an initial laparoscopic approach was abandoned in favor of a thoracoscopic approach after no intraabdominal mass was found. The second patient had an uncomplicated thoracoscopic resection of a similar lesion. To our knowledge, these represent the first intradiaphragmatic pulmonary sequestrations to be resected via a minimally invasive approach. The rarity of these lesions makes definitive diagnosis without operative intervention challenging. Thoracoscopy appears to be a reasonable approach for resection of such intradiaphragmatic lesions.

Congenital cervical cysts, sinuses, and fistulae in pediatric surgery.

Congenital cervical anomalies are essential to consider in the clinical assessment of head and neck masses in children and adults. These lesions can present as palpable cystic masses, infected masses, draining sinuses, or fistulae. Thyroglossal duct cysts are most common, followed by branchial cleft anomalies and dermoid cysts. Other lesions reviewed include median ectopic thyroid, cervical teratomas, and midline cervical clefts. Appropriate diagnosis and management of these lesions requires a thorough understanding of their embryology and anatomy. Correct diagnosis, resolution of infectious issues before definitive therapy, and complete surgical excision are imperative in the prevention of recurrence.

Parapagus conjoined twins with unilateral mesenteric venous outflow.

This case presents a complication of attempted separation of parapagus conjoined twins, related to loss of an intact mesenteric-portal venous axis. Despite known lack of a superior mesenteric artery in the right twin, initial evaluation in the operating room suggested that separation was possible. After hepatic division, however, it became apparent that the mesenteric drainage was not separable; and the operation was aborted. Subsequently, significant growth failure and hypoglycemia were noted in the right twin. The situation was corrected by creating a shunt to reinstitute mesenteric flow to the right twin's liver and separating the twin's mesenteric drainage. One year postoperatively, both twins are independently nourishing themselves and have been free from hospitalization with stable glucoses.

Novel titanium constructs for chest wall reconstruction in children.

PURPOSE: We have previously reported the use of the vertical expandable prosthetic titanium rib (VEPTR) for treatment of thoracic dystrophy. This report describes our experience with this device and other novel titanium constructs for chest wall reconstruction. METHODS: This is a retrospective chart review of all children and adolescents undergoing chest wall reconstruction with titanium constructs between December 2005 and May 2010. RESULTS: Six patients have undergone chest wall reconstruction with VEPTR or other titanium constructs. Four had chest wall resection for primary malignancy, 1 had metastatic chest wall tumor resection, and 1 had congenital chest wall deformity. There were no immediate complications, and all patients have exhibited excellent respiratory function with no scoliosis. CONCLUSIONS: Chest wall reconstruction after tumor resection or for primary chest wall deformities can be effectively accomplished with VEPTR and other customized titanium constructs. Goals should be durable protection of intrathoracic organs and preservation of thoracic volume and function throughout growth. Careful preoperative evaluation and patient-specific planning are important aspects of successful reconstruction.