Integration of Certified Child Life Specialists to Decrease in Periprocedural Benzodiazepine Use: A Pilot Study.

INTRODUCTION: Periprocedural anxiety is common in pediatric patients and is characterized by tension, anxiety, irritability, and autonomic activation. Periprocedural anxiety increases during certain events including admission to the preoperative area, separation from caregivers, induction of anesthesia, and IV placement. A study of children aged 2-12 showed that perioperative anxiety in children may be influenced by high parental anxiety and low sociability of the child. While these are nonmodifiable variables in the perioperative setting, there are numerous ways to ameliorate both parental and patient anxiety including the use of certified child life specialists (CCLSs) to aid in child comfort. In this study, our objective was to evaluate the integration of CCLS in our perioperative setting on the rate of benzodiazepine use. METHODS: We used a prospectively maintained database to identify patients undergoing outpatient elective surgical and radiologic procedures from July 2022 to September 2023 and January 2023 to September 2023 respectively. CCLSs were used to work with appropriately aged children in order to decrease the use of benzodiazepines and reduce possible adverse events associated with their use. RESULTS: A total of 2175 pediatric patients were seen by CCLS in same day surgery from July 2022 to September 2023. During this period, midazolam use decreased by an average of 11.4% (range 6.2%-19.3%). An even greater effect was seen in the radiologic group with 73% reduction. No adverse events were reported during this period. CONCLUSIONS: CCLSs working with age-appropriate patients in the periprocedural setting is a useful adjunct in easing anxiety in pediatric patients, reducing the need for periprocedural benzodiazepine administration and the risk of exposure to unintended side effects.

Survey on Robot-Assisted Surgical Techniques Utilization in US Pediatric Surgery Fellowships.

INTRODUCTION: Robotic technology has transformed both practice and education in many adult surgical specialties; no standardized training guidelines in pediatric surgery currently exist. The purpose of our study was to assess the prevalence of robotic procedures and extent of robotic surgery education in US pediatric surgery fellowships. MATERIALS AND METHODS: A deidentified survey measured utilization of the robot, perception on the utility of the robot, and its incorporation in training among the program directors of Accreditation Council for Graduate Medical Education (ACGME) pediatric surgery fellowships in the United States. RESULTS: Forty-one of the 47 fellowship programs (87%) responded to the survey. While 67% of respondents indicated the presence of a robot in their facility, only 26% reported its utilizing in their surgical practice. Among programs not utilizing the robot, most common reasons provided were lack of clear supportive evidence, increased intraoperative time, and incompatibility of instrument size to pediatric patients. While 58% of program directors believe that there is a future role for robotic surgery in children, only 18% indicated that robotic training should play a part in pediatric surgery education. Consequently, while over 66% of survey respondents received training in robot-assisted surgical technique, only 29% of fellows receive robot-assisted training during their fellowship. CONCLUSIONS: A majority of fellowships have access to a robot, but few utilize the technology in their current practice or as part of training. Further investigation is required into both the technology's potential benefits in the pediatric population and its role in pediatric surgery training.

Asymptomatic HLA-A*02:01-restricted epitopes from herpes simplex virus glycoprotein B preferentially recall polyfunctional CD8+ T cells from seropositive asymptomatic individuals and protect HLA transgenic mice against ocular herpes.

Evidence from C57BL/6 mice suggests that CD8(+) T cells, specific to the immunodominant HSV-1 glycoprotein B (gB) H-2(b)-restricted epitope (gB498-505), protect against ocular herpes infection and disease. However, the possible role of CD8(+) T cells, specific to HLA-restricted gB epitopes, in protective immunity seen in HSV-1-seropositive asymptomatic (ASYMP) healthy individuals (who have never had clinical herpes) remains to be determined. In this study, we used multiple prediction algorithms to identify 10 potential HLA-A*02:01-restricted CD8(+) T cell epitopes from the HSV-1 gB amino acid sequence. Six of these epitopes exhibited high-affinity binding to HLA-A*02:01 molecules. In 10 sequentially studied HLA-A*02:01-positive, HSV-1-seropositive ASYMP individuals, the most frequent, robust, and polyfunctional CD8(+) T cell responses, as assessed by a combination of tetramer, IFN-γ-ELISPOT, CFSE proliferation, CD107a/b cytotoxic degranulation, and multiplex cytokine assays, were directed mainly against epitopes gB342-350 and gB561-569. In contrast, in 10 HLA-A*02:01-positive, HSV-1-seropositive symptomatic (SYMP) individuals (with a history of numerous episodes of recurrent clinical herpes disease) frequent, but less robust, CD8(+) T cell responses were directed mainly against nonoverlapping epitopes (gB183-191 and gB441-449). ASYMP individuals had a significantly higher proportion of HSV-gB-specific CD8(+) T cells expressing CD107a/b degranulation marker and producing effector cytokines IL-2, IFN-γ, and TNF-α than did SYMP individuals. Moreover, immunization of a novel herpes-susceptible HLA-A*02:01 transgenic mouse model with ASYMP epitopes, but not with SYMP epitopes, induced strong CD8(+) T cell-dependent protective immunity against ocular herpes infection and disease. These findings should guide the development of a safe and effective T cell-based herpes vaccine.

Angiodysplasia (vascular malformations) of the colon presenting as an acute abdomen.

Angiodysplasia (vascular malformations) of the colon is extremely rare in children, and, as in adults, present with lower gastrointestinal hemorrhage. Here we report an unusual pediatric case of angiodysplasia of the terminal ileum and cecum presenting as an acute abdomen with radiological features suggestive of lymphoma.

Feasibility of single-incision pediatric endosurgery for treatment of appendicitis in 415 children.

INTRODUCTION: Single-incision pediatric endosurgery (SIPES) has gained popularity for ablative procedures such as appendectomy in many pediatric surgical centers. This study evaluates the outcome of SIPES for treatment of appendicitis in our institution. PATIENTS AND METHODS: After Institutional Review Board approval was obtained, data were prospectively collected on all patients undergoing SIPES appendectomy in our hospital from March 2009 through October 2011. The surgical techniques, operative times, complications, conversion rates, and outcomes were recorded. RESULTS: SIPES appendectomy was attempted in 415 children (mean age, 10.9 years; age range, 1.4-17.9 years; 266 males, 149 females; median weight, 43 kg; weight range, 9.8-146 kg). Intraoperatively, acute appendicitis was found in 298 cases and perforated appendicitis in 79 cases. Thirty-eight patients underwent interval appendectomy. Appendectomy was carried out solely as SIPES in 397 cases (96%). Median operative time was 40±16 minutes (37±16 minutes for fellows [n=284] and 46±15 minutes for residents [n=131]). There were three intraoperative complications, which could be handled during the procedure. Pathologic reports revealed inflammatory changes of the appendix (n=386), other pathology (n=11), and no pathologic change (n=18). Overall, 24 patients (5.8%) were readmitted for intra-abdominal abscess (n=14), umbilical wound infection (n=3), and other reasons (n=7). Twelve patients (2.9%) underwent reoperation: drainage of intra-abdominal abscess (n=8) (3 by the surgeon, 5 by the interventional radiologist), wound drainage (n=3), and right hemicolectomy for carcinoid (n=1). In perforated appendicitis the postoperative intra-abdominal abscess rate was 10 of 79 cases (12.7%), which is similar to the previous report with conventional laparoscopic appendectomy from our institution (13.6%). The wound infection rate (5 of 79 cases [6.3%]) was also similar to the previously report (6.8%) with conventional laparoscopic appendectomy for perforated appendicitis. CONCLUSIONS: Appendectomy can be accomplished successfully and safely using single-incision endosurgery in children with acceptable operative times without leaving any appreciable scar. Additional trocars are infrequently necessary. So far, the intraoperative and postoperative complication rates are comparable to those of triangulated laparoscopic appendectomy.

The herpes simplex virus 1 latency-associated transcript promotes functional exhaustion of virus-specific CD8+ T cells in latently infected trigeminal ganglia: a novel immune evasion mechanism.

Following ocular herpes simplex virus 1 (HSV-1) infection of C57BL/6 mice, HSV-specific (HSV-gB(498-505) tetramer(+)) CD8(+) T cells are induced, selectively retained in latently infected trigeminal ganglia (TG), and appear to decrease HSV-1 reactivation. The HSV-1 latency-associated transcript (LAT) gene, the only viral gene that is abundantly transcribed during latency, increases reactivation. Previously we found that during latency with HSV-1 strain McKrae-derived viruses, more of the total TG resident CD8 T cells expressed markers of exhaustion with LAT(+) virus compared to LAT(-) virus. Here we extend these findings to HSV-1 strain 17syn+-derived LAT(+) and LAT(-) viruses and to a virus expressing just the first 20% of LAT. Thus, the previous findings were not an artifact of HSV-1 strain McKrae, and the LAT function involved mapped to the first 1.5 kb of LAT. Importantly, to our knowledge, we show here for the first time that during LAT(+) virus latency, most of the HSV-1-specific TG resident CD8 T cells were functionally exhausted, as judged by low cytotoxic function and decreased gamma interferon (IFN-γ) and tumor necrosis factor alpha (TNF-α) production. This resulted in LAT(-) TG having more functional HSV-gB(498-505) tetramer(+) CD8(+) T cells compared to LAT(+) TG. In addition, LAT expression, in the absence of other HSV-1 gene products, appeared to be able to directly or indirectly upregulate both PD-L1 and major histocompatibility complex class I (MHC-I) on mouse neuroblastoma cells (Neuro2A). These findings may constitute a novel immune evasion mechanism whereby the HSV-1 LAT directly or indirectly promotes functional exhaustion (i.e., dysfunction) of HSV-specific CD8(+) T cells in latently infected TG, resulting in increased virus reactivation.

Adrenal cortical tumors in children: factors associated with poor outcome.

PURPOSE: The purpose of this study was to evaluate recurrence and survival outcomes in pediatric adrenal cortical neoplasms. METHODS: A 90-year retrospective review of children with adrenal cortical neoplasms was performed using multivariate Cox regression analysis to identify factors associated with recurrence and tumor-related mortality. RESULTS: The evaluable cohort included 29 patients. Twenty-seven underwent resection. Twenty-two (81%) had localized disease, and 5 (19%) had locally advanced disease (all received chemotherapy and 2 of 5 were cured). Two patients presenting with metastatic disease died despite treatment. There were 4 recurrences; all patients died. Tumor-related mortality was 24% (7/29). Kaplan-Meier freedom from recurrence was 85% at 1 year (95% confidence interval, 75%-95%). Multivariate Cox regression revealed that older age (P = .01), higher mitotic rate (P = .005), and necrosis (P < .001) were independent predictors of tumor-related death. Higher mitotic rate (P = .007) and larger tumor size (P = .03) were significant predictors of tumor recurrence. CONCLUSION: Risk factors for poor outcomes in patients with adrenocortical tumors include older age, higher mitotic rate, higher percent necrosis, and larger tumor size. Therefore, the presence of these factors may warrant consideration of adjuvant chemotherapy, even in the absence of advanced disease.

Serial amnioinfusions prevent fetal pulmonary hypoplasia in a large animal model of oligohydramnios.

BACKGROUND/PURPOSE: Severe neonatal pulmonary hypoplasia incurs mortality rates approaching 71% to 95%. We sought to determine the utility of serial amnioinfusions through a subcutaneously implanted intraamniotic catheter to prevent pulmonary hypoplasia in fetal obstructive uropathy. METHODS: Fetal lambs (n = 32) were divided into 3 groups. Group I (n = 12) underwent a sham operation, group II (n = 15) underwent a complete urinary tract obstruction via ligation of the urachus and urethra with a subcutaneous tunneled intraamniotic port-a-cath without amnioinfusions, and group III (n = 5) underwent a creation of a complete urinary tract obstruction with a port-a-cath as described in group II with serial amnioinfusions. Lung tissue was analyzed by lung volume to body weight ratios and stereology. Statistical analysis was performed by analysis of variance and Bonferroni comparisons (P < .05). RESULTS: Obstructed fetuses grossly had smaller lungs than treated and control animals. Lung volume to body weight ratios were statistically significant between groups. Airspace fractions were comparable between groups I and III (average = 0.53 and 0.55, respectively), although both were significantly greater than group II (average = 0.48) (P = .049). CONCLUSIONS: Serial amnioinfusions through an intraamniotic port-a-cath prevented pulmonary hypoplasia in an ovine model of complete obstructive uropathy. The use of an easily accessible device for amnioinfusions may be a viable option to treat oligohydramnios.

Congenital intra-abdominal bilateral juvenile granulosa cell tumors of the testis associated with constitutional loss of material from chromosome 4.

Juvenile granulosa cell tumor (JGCT) is an uncommon gonadal stromal tumor that occurs rarely in the testis. We report a newborn boy with bilateral intra-abdominal JGCT presenting with abdominal distention and respiratory distress at birth. He was taken to the operating room emergently, and 2 large masses connected by gubernacula to the inguinal canals were resected. Associated abnormalities included a constitutional chromosome 4 abnormality, polymicrogyria, and renal cysts. This report describes a rare presentation of JGCT with abdominal compression and expands the literature to include bilateral testicular involvement. Additionally, it is the 1st report of JGCT associated with a chromosome 4 abnormality, highlighting a genetic region that may be important in JGCT development.

Primary lung tumors in children and adolescents: a 90-year experience.

PURPOSE: Primary lung tumors in children are rare. A wide range of histopathologic tumor types occurs. The incidence of these lesions and their outcomes are still largely unknown. This study aims to determine the incidence of different primary lung tumors in children and to contribute data leading to the development of evidence-based treatment models. METHODS: A single institution retrospective review was performed with institutional review board approval. Patients were included if they had primary, nonhematologic lung tumors. Simple squamous papillomas subjected to endoscopic biopsy and not resected, and vascular lesions associated with multisystem lesions, such as hereditary hemorrhagic telangiectasia, were excluded. Medical records and pathologic material for patients from 1918 to 2008 were reviewed. RESULTS: Forty patients were identified (23 boys, 17 girls) with a mean age of 9.6 years (range, 3 months to 19 years). Fourteen distinct histopathologic tumor types were identified. The most common tumor types were carcinoid (8), inflammatory myofibroblastic tumor (7), and pleuropulmonary blastoma (6). Rare pediatric lung tumors including small cell carcinoma, adenocarcinoma, and pulmonary capillary hemangiomatosis were also seen. The mortality rate was 17.5% (7) in our series. Chemotherapy was used in 23% (9) and radiation in 20% (8) of the patients. Of the 33 survivors, 28 had follow-up with a median duration of 29.5 months (mean, 63.2 months; range, 1-471 months). CONCLUSIONS: Primary lung tumors in children are rare and histopathologically diverse. The tumor spectrum involves many types not seen in adults, and unlike adults, patients rarely have a history of exposure to external predisposing factors. Although complete resection remains the standard for treatment of most tumors, addition of adjuvant therapy is dependent on both tumor stage and histopathologic type.

Correlation between prenatal urinary matrix metalloproteinase activity and the degree of kidney damage in a large animal model of congenital obstructive uropathy.

BACKGROUND/PURPOSE: We aimed to determine whether the profile of matrix metalloproteinase (MMP) activity in fetal urine correlates with the degree of kidney damage in the setting of congenital obstructive uropathy. METHODS: Fetal lambs underwent either a sham operation or creation of a complete urinary tract obstruction. Necropsies were performed before term, when urinary MMP profiling was performed by zymography; and kidney damage was assessed histologically by multiple semiquantitative analyses and histomorphometric measurements. RESULTS: There was a significant correlation between inner medullary thickness and MMP-9 (P = .005) and 63-kd MMP-2 (P = .019) activities. In like manner, the only MMPs associated with kidney fibrosis were MMP-9 and 63-kd MMP-2. Matrix metalloproteinase-9 activity was a highly significant independent predictor of the total combined kidney fibrosis score (P < .001) as well as of higher fibrosis grades in each of 6 kidney areas analyzed (all with P < .01). The activity of 63-kd MMP-2 correlated significantly with higher fibrosis in select areas. CONCLUSIONS: In a fetal ovine model, urinary MMP activity correlates with the degree of kidney damage. The presence of MMP-9 (in particular) and that of 63-kd MMP-2 are independent predictors of severity. Prenatal urinary MMP profiling may enhance patient stratification and counseling in the setting of congenital obstructive uropathy.

Prenatal urinary matrix metalloproteinase profiling as a potential diagnostic tool in fetal obstructive uropathy.

BACKGROUND/PURPOSE: The diagnostic evaluation, patient stratification, and prenatal counseling for congenital obstructive uropathy remain sub-optimal. Matrix metalloproteinase (MMP) expression profiles are emerging as a valuable diagnostic tool in assorted disease processes. We sought to determine whether congenital obstructive uropathy impacts MMP expression in fetal urine. METHODS: Fetal lambs (n = 25) were divided in two groups: group I (n = 12) underwent a sham operation and group II (n = 13) underwent creation of a complete urinary tract obstruction. Gelatin zymography panels for 4 MMP species were performed on fetal urine in both groups at comparable times post-operatively. Statistical analysis was by the Fisher's exact test (P < .05). RESULTS: Overall fetal survival was 80% (20/25). A variety of significant differences in MMP expression between the two groups were identified. The following profiles were present only in obstructed animals: any MMP other than MMP-2 (P = .029), including any MMP other than 63 kDa and 65 kDa (P = .009); 2 or more MMPs excluding MMP-2s (0.029); and 3 or more MMPs (P = .029). CONCLUSIONS: Limited matrix metalloproteinase expression is present in the urine of normal ovine fetuses. Fetal obstructive uropathy impacts urinary MMP expression in various distinguishable patterns. Prenatal urinary MMP profiling may become a practical and valuable diagnostic tool in the evaluation of congenital obstructive uropathy.

In utero diagnosis of congenital pyloric atresia in a single twin using MRI and ultrasound.

Congenital pyloric atresia (CPA) is a rare disorder that has traditionally been diagnosed in the postnatal period. With improvements in ultrasound technology and increasing use of MRI, CPA is now diagnosed with increasing accuracy in utero. This also allows for identification of concomitant anomalies, which greatly affects infant survival. In addition, antenatal diagnosis of CPA and associated anomalies allow for family counseling and planning of treatment for the perinatal period including early referral to a center with pediatric surgical services. Here, we present a case of CPA with preoperative diagnosis using ultrasound and MRI.

Childhood pancreatic tumors: a single institution experience.

PURPOSE: The rarity and histopathologic diversity of primary pancreatic neoplasms in children have made it difficult to predict prognosis and to develop optimal management protocols. METHODS: A 90-year (1918-2007), single institution, retrospective review of all patients with neoplastic pancreatic masses was performed. RESULTS: Eighteen patients were identified with 7 distinct histopathologic subtypes. The most common were gastroenteropancreatic neuroendocrine, solid pseudopapillary, and acinar tumors. There were 6 benign and 12 malignant tumors. Six patients had disease outside the pancreas at their initial operation. There were 7 deaths (41%), 2 related to the initial operation, 3 from disease progression, 1 from a small bowel obstruction, and 1 from necrotizing pancreatitis. Five deaths were in patients with extrapancreatic disease found at initial operation. The median duration of follow-up for the 10 survivors was 41 months. CONCLUSION: In adults, pancreatic ductal adenocarcinoma is by far the most common histopathologic subtype, with other subtypes more common in children. Stage is an important prognostic factor. Long-term disease-free survival in childhood pancreatic malignancies is achievable with complete surgical resection, prognosis, and adjuvant treatment, depending on the histopathologic type.

Transoral closure of a perforation of the hypopharynx from blunt trauma.

Hypopharyngeal perforation secondary to blunt trauma is a rare injury. It can be managed operatively or expectantly without clear criteria for either approach. Here, we present a case of a 17-year-old adolescent boy who had a hypopharyngeal tear from direct blunt trauma to the anterior neck received during a high school football game. Physical examination demonstrated cervical crepitus, and neck radiograph revealed air in the retropharyngeal space. Rigid endoscopy diagnosed a 3-cm linear tear in the posterior hypopharynx. The tear was repaired transorally using laparoscopic instruments and visualized by a rigid endoscope, followed by anterior neck dissection and drain placement. Postoperatively, he was kept nil per os and received intravenous antibiotics. He was discharged home and returned to the football field the same fall.

H-type rectovaginal fistula in a patient with bilateral single ectopic ureters.

Congenital H-type rectovaginal fistulas and single ectopic bilateral ureters are each rare malformations. We describe a baby girl with a congenital rectovaginal fistula diagnosed 2 years after correction of single ectopic bilateral ureters. To our knowledge, this is the first association of these entities. Repair of fistula was complicated by recurrence, requiring a second procedure. The recommended operation for this anomaly requires separating the suture lines on the vagina and rectum. The practice of simply oversewing and then buttressing the suture lines is probably not sufficient.

Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation.

PURPOSE: Heterotaxy syndrome (HS) patients often present with congenital heart disease and intestinal malrotation. Controversy exists regarding the management of these patients. Risk of midgut volvulus, morbidity from elective operations, and overall prognosis must be weighed when considering a Ladd procedure on asymptomatic HS/intestinal malrotation patients. METHODS: This is a retrospective review comparing HS and non-heterotaxy syndrome (NHS) patients undergoing a Ladd procedure at Children's Hospital Boston (Mass) from January 1997 to September 2007. RESULTS: Thirty-one HS and 51 NHS patients were identified. After a Ladd procedure, HS patients remained in the hospital 12.9 days, had a 9.7% risk of small bowel obstruction, and a 9.7% in-hospital mortality. If allowed to develop abdominal symptoms, 27% of HS patients with intestinal malrotation had a midgut volvulus at surgery. Intra-HS group comparison demonstrated longer hospital stays in symptomatic patients (P = .01). Mortality was greater in the HS than NHS patients, but deaths were related to cardiac disease and not to the Ladd procedure. CONCLUSIONS: Elective Ladd procedures are well tolerated by HS patients. Given the risk of midgut volvulus and in light of improved survival beyond infanthood, once identified, HS patients with asymptomatic malrotation should be offered a prophylactic Ladd procedure.

Resection of a large, ruptured, undifferentiated (embryonal) sarcoma of the liver in a child: a case report and review of the literature.

BACKGROUND: Undifferentiated (embryonal) sarcoma (UES) of the liver is a malignant hepatic neoplasm accounting for 7% of pediatric hepatic tumors. Current use of multimodal therapy, including chemotherapy and surgery, has greatly improved survival. Tumor rupture is uncommon and, prior to the adjuvant use of sarcoma based chemotherapy regimens, was thought to be poor prognostic sign. CASE PRESENTATION AND DISCUSSION: A 10-year-old girl presented with acute worsening of abdominal pain while being worked up for liver mass. At surgery, she was found to have a ruptured, 20 x 15 x 5 centimeter (cm) UES, the largest reported ruptured UES to be resected primarily. The size and position of her tumor required very aggressive surgery for complete resection. She subsequently received adjuvant chemotherapy and radiation. Twenty-four months following surgery she is doing well with no evidence of disease. CONCLUSION: Despite tumor rupture, complete tumor resection gives the patient the best chance of long-term survival. Aggressive surgery is warranted if the tumor can be completely resected.

Mesenteric lipoblastoma presenting as a segmental volvulus.

Mesenteric lipoblastoma is a rare tumor and, its presentation as a bowel obstruction with possible midgut volvulus has only been reported once before. A 7-year-old girl presented with nausea and vomiting but a benign abdominal examination. Upper gastrointestinal contrast study demonstrated possible malrotation with midgut volvulus. During emergency laparotomy, segmental small bowel volvulus secondary to a large mesenteric lipoblastoma was found. The lipoblastoma was resected with a segment of small bowel. Resection margins were negative for tumor, and the patient is doing well with no evidence of recurrence.

Use of a distal arteriovenous fistula in a complex vascular reconstruction after resection of an osteosarcoma in a high-risk child.

Osteosarcoma is the most common osseous malignancy of childhood. Vascular reconstruction during resection is uncommon. However, when required, thrombosis is a major cause of failed limb salvage surgery. A 19-year-old patient required complex vascular reconstruction in conjunction with lower extremity osteosarcoma resection. Preoperatively, his case was complicated by an ipsilateral popliteal deep vein thrombosis presenting during neoadjuvant chemotherapy. At the time of resection, to increase vascular graft flow, a distal arteriovenous fistula was created between the posterior tibial artery and the greater saphenous vein. This case illustrates a patient with a complex extirpation procedure requiring concomitant vascular reconstruction. This technique should be considered when limb salvage with vascular resection is being attempted in a high-risk patient.