Assessing Alternative Approaches for Wound Closure in a National Pediatric Learning Health System.

INTRODUCTION: Our objective was to perform a feasibility study using real-world data from a learning health system (LHS) to describe current practice patterns of wound closure and explore differences in outcomes associated with the use of tissue adhesives and other methods of wound closure in the pediatric surgical population to inform a potentially large study. METHODS: A multi-institutional cross-sectional study was performed of a random sample of patients <18 y-old who underwent laparoscopic appendectomy, open or laparoscopic inguinal hernia repair, umbilical hernia repair, or repair of traumatic laceration from January 1, 2019, to December 31, 2019. Sociodemographic and operative characteristics were obtained from 6 PEDSnet (a national pediatric LHS) children's hospitals and OneFlorida Clinical Research Consortium (a PCORnet collaboration across 14 academic health systems). Additional clinical data elements were collected via chart review. RESULTS: Of the 692 patients included, 182 (26.3%) had appendectomies, 155 (22.4%) inguinal hernia repairs, 163 (23.6%) umbilical hernia repairs, and 192 (27.8%) traumatic lacerations. Of the 500 surgical incisions, sutures with tissue adhesives were the most frequently used (n = 211, 42.2%), followed by sutures with adhesive strips (n = 176, 35.2%), and sutures only (n = 72, 14.4%). Most traumatic lacerations were repaired with sutures only (n = 127, 64.5%). The overall wound-related complication rate was 3.0% and resumption of normal activities was recommended at a median of 14 d (interquartile ranges 14-14). CONCLUSIONS: The LHS represents an efficient tool to identify cohorts of pediatric surgical patients to perform comparative effectiveness research using real-world data to support medical and surgical products/devices in children.

Long-term outcomes of antegrade continence enema in children with chronic encopresis and incontinence: what is the optimal flush to use?

PURPOSE: Severe constipation and encopresis are significant problems in the pediatric population. Medical management succeeds in 50-70%; however, surgical considerations are necessary for the remainder such as the antegrade continence enema (ACE). The purpose of this study is to assess the long-term outcomes following the ACE procedure. METHODS: All patients undergoing an ACE over a 14-year period were included. Data on clinical conditions, treatments, and outcomes were collected. A successful outcome was defined as remaining clean with ≤ 1 accident per week. Comparative data were analyzed using the Fisher's exact test, Mann-Whitney U test, or Student's t test. RESULTS: There were 42 ACE patients, and overall, 79% had improvement in their bowel regimens. Encopresis rates decreased from 79 to 5% (P < 0.001). Admissions for cleanouts decreased from 52 to 19% (P = 0.003). All cases of Hirschsprung's, functional constipation and spina bifida were successful. Rates of success varied for other diseases such as slow-transit constipation (60%) and cerebral palsy (33%). A majority (85%) required a change in the enema composition for improvement. CONCLUSION: In our study, ACE reduced soiling, constipation, and need for fecal disimpaction. Higher volume saline flushes used once a day was the optimal solution and most preferred option. LEVEL OF EVIDENCE: Level 4 (retrospective case series or cohort).

Diaphragm Pacing as a Rehabilitative Tool for Patients With Pompe Disease Who Are Ventilator-Dependent: Case Series.

BACKGROUND AND PURPOSE: Pompe disease is an inherited disorder notable for severe, progressive ventilatory compromise. Although ventilatory failure has been attributed to myofiber dysfunction secondary to diaphragmatic glycogen accumulation, neural involvement of the phrenic motor system is also a prominent feature. Direct diaphragm pacing supplements respiratory function in other disorders of the phrenic motor system. Accordingly, it is hypothesized that augmented neuromuscular activity via diaphragm pacing would promote weaning from mechanical ventilation in patients with Pompe disease who are unresponsive to conventional, muscle-directed treatments. CASE DESCRIPTION: Three patients with Pompe disease developed diaphragm paresis that resulted in chronic mechanical ventilation dependence. After preoperative inspiratory muscle strengthening exercises failed to improve function, fine-wire pacing electrodes were laparoscopically implanted into the diaphragm. Diaphragm conditioning was initiated the first postoperative week and consisted of gradual increases in stimulation parameters, lengthening of stimulation sessions, and ventilator weaning. Ventilation and intramuscular electromyographic activity were recorded periodically during conditioning to quantify diaphragm neuromuscular function. OUTCOMES: During paced breathing without mechanical ventilation, tidal volumes increased, and 2 patients were weaned from daytime ventilator dependence within the first 3 months of pacing, which has been sustained over the long-term. A third patient reduced reliance on daytime ventilation, but weaning was delayed by malacia of the large airways. In all patients, pacing appeared to facilitate spontaneous phrenic motor unit activity during independent breathing without ventilator or pacer support. DISCUSSION: The findings are consistent with the view that diaphragm pacing has potential rehabilitative value to reduce reliance on mechanical ventilation in people with Pompe disease, but further study is needed. Diaphragm pacing represents a paradigm shift in the management of respiratory insufficiency for Pompe disease that warrants further controlled examination.

Long-term outcomes of gastric electrical stimulation in children with gastroparesis.

BACKGROUND: Gastric electrical stimulation (GES) has been used in adults with gastroparesis. However its use has been limited in children. We describe the largest experience with GES in children with long-term outcomes. METHODS: Data were collected on children who underwent GES over a 10-year period. Data regarding demographics, medical history, hospital course, and outcomes were collected and analyzed. Symptom scores (validated Likert scores) were compared using a paired Student's t test. RESULTS: Overall, 97 patients underwent GES, and a majority were teenage Caucasian girls. Ninety-six had temporary GES (tGES), and 66 had improvement in their symptoms. A total of 67 underwent permanent implantation (pGES), and there was significant reduction in all individual symptoms (p<0.001) as well as the total symptom score (TSS) (p<0.0001) at 1, 6, 12, and >12 months. Recurrence of symptoms leading to device removal occurred in 7 cases. Forty-one patients had continued improvement in symptoms for over 12 months, with a mean follow-up of 3.5 years (range 1-9 years). CONCLUSIONS: This study represents the largest experience of systematic application of GES in children. GES is a safe and effective therapy for selected children with intractable GP with continued symptomatic improvement at 1 year and beyond.

Gastroparesis in children.

PURPOSE OF REVIEW: Gastroparesis is a disorder with delayed gastric emptying in the absence of mechanical obstruction. It is one of the more common dysmotilities that occur in the gastrointestinal tract, and is thought to primarily affect adults. Pediatric cases of gastroparesis were considered rare; however, recent evidence suggests growing recognition in children and adolescents. Therefore, it is important for the pediatric caregiver to understand the condition and the treatment options available. RECENT FINDINGS: The majority of patients are women, and presentation is usually with symptoms of persistent nausea, emesis, postprandial pain and bloating, and early satiety. Weight loss may occur in some cases, though this is not universal. The majority of cases are idiopathic, with diabetes mellitus the second most common cause. SUMMARY: Treatment consists of symptomatic relief with medication to counteract the nausea, emesis, pain, bloating, gastroesophageal reflux, early satiety, and improve gastric emptying. Dietary modification is also used with small meals and avoidance of high fiber and fat-containing foods. Recalcitrant cases of gastroparesis require the use of additional approaches such as jejunal feeds, intrapyloric botulinum toxin, gastric emptying procedures such as pyloroplasty, and gastric electrical stimulation. We will review these options in this article.

Variation in bowel preparation among pediatric surgeons for elective colorectal surgery: A problem of equipoise or a knowledge gap of the available clinical evidence?

PURPOSE: Despite rigorous data from adult literature demonstrating that oral antibiotics (OA) reduce infectious complications and mechanical bowel preparation (MBP) alone does not, MBP alone remains the preferred approach among pediatric surgeons. We aimed to explore the nature of this discrepancy through a survey of the American Pediatric Surgical Association membership. METHODS: Surgeons were queried for their choice of bowel preparation, factors influencing their practice, and their impression of the strength and relevance of the adult literature to pediatric practice. RESULTS: Surgeons who used MBP alone (31%) cited a reduction in stool burden and infectious complications as important factors, whereas surgeons choosing not to use OA (70%) reported a lack of benefit in reducing infectious complications as the primary reason. Although 53% of surgeons reported that evidence from adult literature was the most important influence, 73% of surgeons reported there was poor evidence supporting the use of OA (±MBP), and only 25% used a preparation supported by adult randomized data. CONCLUSIONS: Wide variation exists among pediatric surgeons in the perceived utility of MBP and OA. Although the majority of pediatric surgeons cited the adult literature as the strongest influence on their practice, this is not consistent with stated perceptions or practice.

Use of mechanical bowel preparation and oral antibiotics for elective colorectal procedures in children: is current practice evidence-based?

PURPOSE: It is well established through randomized trials that oral antibiotics given with or without a mechanical bowel preparation (MBP) prior to colorectal procedures reduce complications, while MBP given alone provides no benefit. We aimed to characterize trends surrounding bowel preparation in children and determine whether contemporary practice is evidence-based. METHODS: Retrospective analysis of patients undergoing colorectal procedures at 42 children's hospitals (1/2/2007-12/31/2011) was performed. Patients were analyzed for diagnosis, pre-admission status, and inpatient bowel preparation. Bowel preparation was considered evidence-based if oral antibiotics were utilized with or without a MBP. RESULTS: 49% of all patients were pre-admitted (n=5,473), and the most common diagnoses were anorectal malformations (55%), inflammatory bowel disease (26%), and Hirschsprung's Disease (19%). The most common preparation approaches were MBP alone (54.3%), MBP+oral antibiotics (18.8%), and oral antibiotics alone (4.2%), although significant variation was found in hospital-specific rates for each approach (MBP alone: 0-96.1%, MBP+oral antibiotics: 0-83.6%, orals alone: 0-91.6%, p<0.0001). Only 22.9% of all patients received an evidence-based preparation (range by hospital: 0-92.3%, p<0.0001), and this rate decreased significantly during the five-year study period (27.6% in 2007 vs. 17.3% in 2011, p<0.0001). CONCLUSION: According to the best available clinical evidence, less than a quarter of all children pre-admitted for elective colorectal procedures receive a bowel preparation proven to reduce infectious complications.

Gastric electrical stimulation for abdominal pain in patients with symptoms of gastroparesis.

Abdominal pain physiology may be better understood studying electrophysiology, histology, and symptom scores in patients with the symptoms of gastroparesis (Gp) treated with gastric electrical stimulation (GES). Ninety-five Gp patients' symptoms were recorded at baseline and during temporary and permanent GES. Gastric-emptying times and cutaneous, mucosal, and serosal electrogastrograms were obtained. S100-stained, full-thickness gastric biopsies were compared with autopsy controls. Sixty-eight patients reported severe pain at baseline. Severe pain patients' mean pain scores decreased with temporary GES from 3.62 to 1.29 (P < 0.001) and nonsevere pain from 1.26 to 0.67 (P = 0.01). With permanent GES, severe mean pain scores fell to 2.30 (P < 0.001); nonsevere pain changed to 1.60 (P = 0.221). Mean follow-up was 275 days. Mean cutaneous, mucosal, and serosal frequencies and frequency-to-amplitude ratios were markedly higher than literature controls. For patients with Gp overall and subdivided by etiology and severity of pain, S-100 neuronal fibers were significantly reduced in both muscularis propria layers. GES improved severe pain associated with symptoms of Gp. This severe pain is associated with abnormal electrogastrographic activity and loss of S100 neuronal fibers in the stomach's inner and outer muscularis propria and, therefore, could be the result of gastric neuropathy.

"Pulling the plug"--management of meconium plug syndrome in neonates.

BACKGROUND: The significance of meconium plug syndrome (MPS) is unclear but has been associated with Hirschsprung's disease and magnesium tocolysis. We reviewed our experience to attempt to identify any potential association with these conditions and to review our outcomes. METHODS: Using the International Classification of Diseases, Ninth revision, code for meconium obstruction, patient charts were identified during the 1998-2008 period. A total of 61 cases of MPS were found, after excluding 7 of meconium ileus. Data regarding the hospital course and outcomes were collected and analyzed. RESULTS: Approximately 30% of patients had spontaneous resolution of the meconium plug without any treatment. Of those patients requiring treatment, contrast barium enema was used, with 97% success. Only 2 patients required surgical intervention owing to worsening distension and subsequent peritonitis. When we stratified the patients according to gestational age of >36 and <36 wk, contrast barium enemas were performed 2.2 ± 1.8 versus 8.6 ± 7.8 d after birth (P = 0.003), respectively, and the lower gestational age patients had a longer length of stay. Contrast barium enema was still successful in 94% of patients with a gestational age of <36 wk. Magnesium tocolysis was noted in 16% of the cases, and Hirschsprung's disease was only found in 3.2% of patients. CONCLUSIONS: Patients with MPS have excellent outcomes, independent of gestational age. Contrast barium enema remains the initial diagnostic and treatment of choice for patients with MPS. Also, although previous reports have shown a link between magnesium tocolysis and Hirschsprung's disease with MPS, our experience suggests otherwise.

Diminishing role of contrast enema in simple meconium ileus.

PURPOSE: Contrast enema is the initial study of choice for simple meconium ileus to confirm diagnosis and to relieve obstruction. Despite favorable historically published results, our clinical impression suggests decreased effectiveness of the contrast enema resulting in more surgical interventions in contemporary practice. METHODS: A retrospective multiinstitutional review for a 12-year period was conducted for neonates diagnosed with meconium ileus by contrast enema. The neonates were divided into 2 groups-historic group (HG = before 2002) and contemporary group (CG = after 2002). T test was used for comparison of continuous variables and chi(2) for categorical data. RESULTS: Thirty-seven total patients were identified (21 females and 16 males). Obstruction was relieved in 8 neonates (22% overall success rate). Average enema attempt per patient was decreased in the CG group compared to HG (1.4 vs 1.9). The success rate in the CG group was 5.5% (1/18) compared to 39% (7/18) in HG. CONCLUSIONS: In this review, success of contrast enema for relief of meconium ileus has significantly decreased over time. These findings may be because of reluctance to repeat enemas, change in radiologist experience, or use of contrast agent. As a result, higher rates of operative intervention are now observed. In stable patients, surgeons should recommend repeat enemas before exploration.

Gastric electrical stimulation for children with intractable nausea and gastroparesis.

PURPOSE: Gastric electrical stimulation (GES) has been performed in adults as a treatment of refractory nausea and vomiting in patients who have failed medical treatment, but has not been used in children. METHODS: Nine patients with chronic nausea and vomiting with a mean age of 14 years were evaluated for temporary GES. All 9 patients subsequently underwent placement of a temporary followed by permanent GES device. Symptoms were recorded at baseline, after temporary GES, and then after permanent GES using a Likert scale for gastroparesis. Statistical analysis was performed using a paired Student's t test. RESULTS: At baseline, all patients were symptomatic and most had delayed solid gastric emptying. As a group, there was a significant improvement in combined symptoms score (P = .04), nausea (P = .039), and vomiting (P = .0016). Gastric emptying and electrogastrogram values did not change significantly. Follow-up ranged from 8 to 42 months, with 7 of the 9 patients reporting sustained improvement in symptoms and improved quality of life. CONCLUSIONS: Gastric electrical stimulation can be successfully applied to adolescents with intractable nausea and gastroparesis symptoms who fail medical therapy. There is a significant improvement in symptoms over a prolonged period, and there are no adverse effects of the GES. Long-term efficacy of this therapy in children needs to be established.

Congenital short-gut syndrome.

A case of congenital short gut is reported in a 4-month-old boy presenting with failure to thrive. Upper gastrointestinal examination (UGI) with small bowel follow-through (SBFT) demonstrated dilation of the duodenum and jejunum, with rapid transit to rectum. On barium enema (BE), rapid transit of barium was noted to the dilated proximal small bowel seen on UGI/SBFT. Neither study delineated the ileocecal region, and the overall length of bowel appeared short. The diagnosis of congenital short gut was confirmed at surgery. Congenital short-gut syndrome is a rare entity. The diagnosis can be suggested by imaging, but is usually confirmed operatively.