The in-utero diagnosis of choledochal cyst: can postnatal imaging predict benefit from early surgical intervention?

BACKGROUND: Infants prenatally suspected of having a choledochal cyst (CDC) typically undergo ultrasound imaging shortly after birth. This study sought to evaluate features on the initial postnatal ultrasound (IPU) that could identify newborns at risk for early complications. METHODS: Following IRB approval, patients from four US fetal centers with prenatal suspicion for CDC and postnatal imaging from 2000 to 2017 were reviewed. Imaging and clinical courses were assessed. RESULTS: Forty-two patients had prenatal ultrasounds suspicious for CDC. Nineteen (45.2%) were excluded due to diagnostic revision (n = 9), cyst resolution (n = 5), lack of IPU measurements (n = 3), or lack of follow-up (n = 2). The 23 remaining patients were included in the study. Of these, five (21.7%) developed symptoms at a median age of 16.5 days (IQR 16-19 days), and 18 (78.3%) remained asymptomatic throughout the first year after birth. Five patients (21.7%) had cysts ≥ 4.5 cm on IPU (Symptomatic: n = 3; Asymptomatic: n = 2). Eighteen patients (78.3%) had cysts < 4.5 cm on IPU (Symptomatic: n = 2; Asymptomatic: n = 16). An IPU cyst size ≥ 4.5 cm was associated with neonatal symptom manifestation (p = 0.048), with 88.9% specificity (95% CI 65.3-98.6%) and 60% sensitivity (95% CI 14.7-94.7%). CONCLUSIONS: In newborns with prenatally diagnosed CDC, a cyst size ≥ 4.5 cm on IPU is associated with symptom development during the first month after birth and therefore early cyst excision is recommended.

Role of Nerve and Muscle Biopsies in Pediatric Patients in the Era of Genetic Testing.

BACKGROUND: An operative biopsy is an important component in the diagnosis and treatment of neuromuscular disorders (NMDs). However, recent advances in molecular genetics suggest less invasive genetic testing should be the initial approach. The purpose of our study was to demonstrate the diagnostic value of muscle or nerve biopsy within the pediatric population at a pediatric academic center and offer recommendations for genetic testing in relation to biopsy to achieve the highest diagnostic yield. METHODS: Following institutional review board approval, we retrospectively reviewed the electronic medical record of 221 pediatric patients who underwent muscle and/or nerve biopsy for suspicion of NMD from January 2007 to March 2018. Demographics, family history, clinical presentations, genetic testing results, pathology results, anesthesia complications, clinical diagnoses, and clinic follow-up data were collected. Chi-square analysis was done for statistical significance. RESULTS: A total of 220 underwent muscle biopsy, and 15 underwent nerve biopsy. Not all patients received genetic testing. The average age at biopsy was 7.7 y. Biopsy revealed significant histologic abnormalities in 62.9% (139), directly leading to a specific clinical diagnosis in 33.9% (75). When genetic testing was done before biopsy, definite pathogenic variants were found in 7.6% (9). When genetic testing was done after biopsy, definite pathogenic variants were found in 45.0% (27). Genetic testing yield for pathogenic variants was higher when done after biopsy (P value < 0.00001). CONCLUSIONS: Muscle and nerve biopsies may provide significant diagnostic value. Biopsy helped to rule in or out NMD and guide genetic testing. Our data suggest NMD genetic testing yield was higher when done after biopsy.

Correlation of payor status and pediatric transfer for acute appendicitis.

BACKGROUND: Tertiary referral centers provide specialty and critical care for patients presenting to hospitals that lack these resources. There is a notion among tertiary centers that outside hospitals are more likely to transfer uninsured or underinsured patients. We examined funding status of patients transferred to our tertiary pediatric hospital for surgical management of appendicitis, hypothesizing that transferred patients were more likely to have unfavorable coverage. MATERIALS AND METHODS: The electronic medical record was queried for all cases of laparoscopic appendectomy at our hospital between 2011 and 2015. Insurance was grouped into three categories: commercial, Medicaid/Children's Health Insurance Plan, or none. Transferred patients were compared to patients who presented directly. RESULTS: A total of 5758 patients underwent laparoscopic appendectomy during the study period, of which 1683 (29.2%) were transfer patients. Transfer patients were more likely to be older, with a median age of 10.5 y versus 9.8 y in nontransferred patients (P ≤ 0.0001), and were more likely to be identified as non-Hispanic (50.0% versus 36.5%; P ≤ 0.0001). Insurance coverage was similar between groups. However, subgroup analysis of the hospitals that most frequently used our transfer services revealed a trend to transfer a higher proportion of Medicaid/Children's Health Insurance Plan patients. CONCLUSIONS: Overall, pediatric patients transferred for laparoscopic appendectomy had similar insurance coverage to patients admitted directly, but subgroup analysis shows that not all centers follow this trend. Transfer patients were more frequently older and non-Hispanic. This builds upon the existing literature regarding the correlation of funding and transfer practices and highlights the need for additional research in this area.

Management of nonparasitic splenic cysts in children.

BACKGROUND: The management of nonparasitic splenic cysts in children is unclear. Options include observation, cystectomy, partial or total splenectomy and percutaneous aspiration with and without sclerotherapy. The aim of this study is to assess the outcomes of these interventions at a children's hospital. MATERIALS AND METHODS: A retrospective review of patients aged <18 y with splenic cysts over 7 y was performed. Demographics, mode of intervention, and outcome data were collected. RESULTS: Forty-two patients were identified and their initial management was as follows: 32 patients were observed and 10 underwent intervention (four aspiration and sclerotherapy and six resection). Age (y) was higher for intervention patients than observation patients (P = 0.004), as was the cyst size (P < 0.001). Incidental finding was the most common presentation in observation patients (n = 30; 94%) and abdominal pain for intervention groups: aspiration and sclerotherapy (n = 3; 75%) and resection (n = 5; 83%). Two patients failed observation and required aspiration and sclerotherapy due to persistence of symptoms or size increase. Median number of aspiration with and without sclerotherapy interventions was three (range 1-5). All six patients had persistence, with two requiring surgical resection due to symptomatic persistence. Surgical procedures included laparoscopic cystectomy (n = 3), laparoscopic partial (n = 2) or complete splenectomy (n = 1), and/or open splenectomy (n = 2). One laparoscopic cystectomy patient had persistence but the other two had no follow-up imaging. Partial and total splenectomy patients had no recurrence and/or persistence. CONCLUSIONS: Observation is an appropriate management strategy for small asymptomatic splenic cysts. Aspiration with and without sclerotherapy and laparoscopic cystectomy are associated with higher rates of recurrence; thus, partial splenectomy may provide the best balance of recurrence and spleen preservation.

Radiation exposure and safety practices during pediatric central line placement.

PURPOSE: Pediatric surgeons routinely use fluoroscopy for central venous line (CVL) placement. We examined radiation safety practices and patient/surgeon exposure during fluoroscopic CVL. METHODS: Fluoroscopic CVL procedures performed by 11 pediatric surgeons in 2012 were reviewed. Fluoroscopic time (FT), patient exposure (mGy), and procedural data were collected. Anthropomorphic phantom simulations were used to calculate scatter and dose (mSv). Surgeons were surveyed regarding safety practices. RESULTS: 386 procedures were reviewed. Median FT was 12.8 seconds. Median patient estimated effective dose was 0.13 mSv. Median annual FT per surgeon was 15.4 minutes. Simulations showed no significant difference (p=0.14) between reported exposures (median 3.5 mGy/minute) and the modeled regression exposures from the C-arm default mode (median 3.4 mGy/minute). Median calculated surgeon exposure was 1.5 mGy/year. Eight of 11 surgeons responded to the survey. Only three reported 100% lead protection and frequent dosimeter use. CONCLUSION: We found nonstandard radiation training, safety practices, and dose monitoring for the 11 surgeons. Based on simulations, the C-arm default setting was typically used instead of low dose. While most CVL procedures have low patient/surgeon doses, every effort should be used to minimize patient and occupational exposure, suggesting the need for formal hands-on training for nonradiologist providers using fluoroscopy.

Single-incision laparoscopic surgery in children: a randomized control trial of acute appendicitis.

BACKGROUND: Single-incision laparoscopic surgery (SILS) is a novel area of minimally invasive surgery that uses a single incision to minimize all ports to one site. The end result is an incision that can be strategically placed in the umbilicus for a perceived scarless abdomen. The authors rationalized that a randomized controlled trial was important given the rapid popularization of this approach. METHODS: An institutional review board-approved prospective randomized trial compared patients undergoing SILS (SILS-A) and conventional laparoscopic (LAP-A) appendectomy at a free-standing children's hospital during a median follow-up period of 2.2 years. RESULTS: A total of 50 patients (50 % boys and 67 % Hispanics) were randomized equally to SILS-A and LAP-A. The patients ranged in age from 3 to 15 years without a difference between the two groups. Half (50 %) of these patients were younger than 8 years. The technique for SILS-A involved a single supraumbilical curvilinear skin incision with three fascial incisions. Ports were inserted to varying depths to minimize restriction of instrument movement. Coaxial visualization was improved by the use of a 30° scope. To achieve technical comparability with the LAP-A, a stapler device was used, which required upsizing a 5 mm port to a 12 mm port. The mean duration of the operation was 46.8 ± 3.7 min (range, 22-120 min) compared with 34.8 ± 2.5 min (range, 18-77 min) for standard LAP-A (p = 0.010). No conversions occurred, and the two groups did not differ in hospital length of stay. The postoperative complications consisted of one wound seroma in the SILS-A group (nonsignificant difference), and no hernias were seen. No difference in readmissions, diet tolerance, fever, or postoperative pain was noted between the two groups. CONCLUSIONS: The findings show the SILS approach to be feasible in the pediatric population despite the limited abdominal domain in younger children. Although SILS operating room times currently are longer than for LAP-A, they are comparable, and no other outcomes differed appreciably between the two techniques at the time of hospitalization or during the follow-up period.