Matthew-Wood Syndrome in Monochorionic, Diamnionic Twins.

Matthew-Wood syndrome represents a rare genetic disorder characterized by diaphragmatic defects, pulmonary hypoplasia, micro- or anophthalmia, and cardiac defects. Most cases are lethal with very few infants living beyond a few years of life. Siblings with this diagnosis have been reported but never twins. In this article, we provided a review and discussion of this syndrome following its presentation in monochorionic, diamnionic twin females.

Outcomes of laparoscopic and open ventriculoperitoneal shunt placement.

OBJECTIVE: Ventriculoperitoneal (VP) shunt placement requires a concurrent abdominal procedure. For peritoneal access laparoscopic or open approach may be utilized. Our aim was to compare patient/procedure characteristics and outcomes by peritoneal approach for VP shunts in children. METHODS: NSQIP-Pediatric procedure targeted cerebral spinal fluid shunt Participant Use Data Files from 2016 to 2020 were queried. Patients were grouped into laparoscopic vs open abdominal approach. Patient demographics, procedure characteristics and 30-day outcomes were compared. RESULTS: 7742 NSQIP-Pediatric patients underwent VP shunt placement. Patients undergoing laparoscopic approach were older and required less preoperative support. Mean operative time was longer with laparoscopy (mean(SD): 74.2(48.1) vs. 64.6(39) minutes, p â€‹< â€‹0.0001) but had shorter hospital LOS. There was no difference in SSI, readmissions, or reoperation rates. CONCLUSION: Patients undergoing laparoscopy for distal VP shunts are older with less support needs preoperatively. While laparoscopic approach had a shorter hospital LOS, there was no demonstratable difference in SSI, readmissions or reoperations between approaches. Further studies are needed to assess long-term outcomes.

A rare cause of infantile achalasia: GMPPA-congenital disorder of glycosylation with two novel compound heterozygous variants.

Achalasia is rare in the pediatric population and should prompt clinicians to consider genetic disorders associated with this condition. While AAA syndrome (also known as Allgrove or Triple A syndrome) is commonly considered, GMPPA-congenital disorder of glycosylation (CDG) should also be in the differential diagnosis. We report a 9-month-old female born to nonconsanguineous parents with achalasia and alacrima found to have two novel compound heterozygous variants in the GMPPA gene associated with GMPPA-CDG. This rare disorder is commonly associated with developmental delay and intellectual disability. We discuss management of this disorder including the importance of confirming a genetic diagnosis and summarize reported cases.

Laparoscopic Co-surgeon Ventriculoperitoneal Shunt Placement Versus Single Surgeon Mini Laparotomy.

INTRODUCTION: Ventriculoperitoneal (VP) shunt placement is one of the most common treatments for pediatric hydrocephalus. However, device failures often occur, requiring operative revision of either the intraventricular or intraperitoneal shunt catheters. Historically, shunt placement was performed via laparotomy, but there has been a trend towards laparoscopic-assisted placement of the intraperitoneal portion of the shunt. We examined the outcomes of laparoscopic-assisted versus open VP shunt placement utilizing a local institutional retrospective review. METHODS: Single institution 2012-2017 retrospective review of all cases was performed. Patients were divided into two groups - laparoscopic and open. Thirty-day outcomes, patient age, surgery performed, surgical control time (SCT), length of stay (LOS), and readmission were analyzed.  Results: Cohort analysis inclusion criteria included 188 patients. The cohort analysis showed both decreased laparoscopic-assisted SCT (56.4 vs 32.1 min, p<0.0001) and postop complications (16.7% vs 7.1%, p<0.07). There was no significant difference in surgical site infection or readmission rates.  Conclusion: Local analysis show advantages for laparoscopic-assisted VP shunt placement over open single surgeon techniques with decreased SCT, LOS, and unplanned interventions.

Novel Way to Improve Satisfaction, Comprehension, and Anxiety in Caregivers: A Randomized Trial Exploring the Use of Comprehensive, Illustrated Children's Books for Pediatric Surgical Populations.

BACKGROUND: Surgery generates anxiety and stress, which can negatively impact informed consent and postoperative outcomes. This study assessed whether educational, illustrated children's books improve comprehension, satisfaction, and anxiety of caregivers in pediatric surgical populations. METHODS: A prospective randomized trial was initiated at a tertiary care children's hospital. All patients ≤ 18 years old with caregiver and diagnosis of 1) uncomplicated appendicitis (English or Spanish speaking); 2) ruptured appendicitis; 3) pyloric stenosis; 4) need for gastrostomy tube; or 5) umbilical hernia were eligible. Conventional consent was obtained followed by completion of 17 validated survey questions addressing apprehension, satisfaction, and comprehension. Randomization (2:1) occurred after consent and before operative intervention with the experimental group (EG) receiving an illustrated comprehensive children's book outlining anatomy, pathophysiology, hospital course, and postoperative care. A second identical survey was completed before discharge. Primary outcomes were caregiver apprehension, satisfaction, and comprehension. RESULTS: Eighty caregivers were included (55: EG, 25: control group [CG]). There were no significant differences in patient or caregiver demographics between groups. The baseline survey demonstrated no difference in comprehension, satisfaction, or apprehension between groups (all p values NS). After intervention, EG had significant improvement in 14 of 17 questions compared with CG (all p < 0.05). When tabulated by content, there was significant improvement in comprehension (p = 0.0009), satisfaction (p < 0.0001), and apprehension (p < 0.0001). CONCLUSION: The use of illustrated educational children's books to explain pathophysiology and surgical care is a novel method to improve comprehension, satisfaction, and anxiety of caregivers. This could benefit informed consent, understanding, and postoperative outcomes.

Home intravenous versus oral antibiotics following appendectomy for perforated appendicitis in children: a randomized controlled trial.

PURPOSE: To compare the effect of home intravenous (IV) versus oral antibiotic therapy on complication rates and resource utilization following appendectomy for perforated appendicitis. METHODS: This was a randomized controlled trial of patients aged 4-17 with surgically treated perforated appendicitis from January 2011 to November 2013. Perforation was defined intraoperatively and divided into three grades: I-contained perforation, II-localized contamination to right gutter/pelvis, and III-diffuse contamination. Patients were randomized to complete a ten-day course of home antibiotic therapy with either IV ertapenem or oral amoxicillin-clavulanate. Thirty-day postoperative complication rates including abscess, readmission, wound infection, and charges were compared. RESULTS: Eighty-two patients were enrolled. Forty four (54%) were randomized to the IV group and 38 (46%) to the oral group. IV patients were older (12.3 ± 3.6 versus 10.1 ± 3.6, p < 0.05) with higher BMI (20.9 ± 5.8 versus 17.9 ± 3.5, p < 0.05). There were no differences in gender, comorbidities, or perforation grade (I-20.4% vs. 26.3%, II-36.4% vs. 34.2%, III-43.2% vs. 39.5%, all p > 0.05). Comparing IV to oral, there was no difference in length of stay (4.4 ± 1.5 versus 4.4 ± 2.0 days, p > 0.05), postoperative abscess rate (11.6% vs. 8.1%, p > 0.05), or readmission rate (14.0% vs. 16.2%, p > 0.05). Hospital and outpatient charges were higher in the IV group (p < 0.0001). CONCLUSION: Oral antibiotics had equivalent outcomes and incurred fewer charges than IV antibiotics following appendectomy for perforated appendicitis.

Thoracoscopic Versus Open Congenital Diaphragmatic Hernia Repair: Single Tertiary Center Review.

BACKGROUND: Congenital diaphragmatic hernia (CDH) can be repaired open or through thoracoscopy. Thoracoscopic CDH repair could improve cosmesis and avoid the complications of laparotomy, but may have higher recurrence rates. The purpose of this study was to examine the outcomes of thoracoscopic versus open CDH repair, with regard to recurrence, perioperative parameters, and postoperative complications. METHODS: We performed a retrospective review of open versus thoracoscopic CDH repairs over an 8.5-year period. The primary outcome was hernia recurrence. Secondary outcomes included intraoperative partial pressure of carbon dioxide (pCO2) levels, length of stay, and postoperative complications. All statistical analyses were performed using standard statistical methods. RESULTS: A total of 54 infants underwent CDH repair during the study period, of whom 25 underwent successful thoracoscopic repair. Two patients who had undergone open repair developed recurrent diaphragmatic hernias (recurrence rate 3.7%). Operative time and intraoperative pCO2 levels did not differ between groups. Length of stay was shorter in the thoracoscopic cohort. Four patients in the open cohort developed ventral hernias and five developed bowel obstructions during follow-up. No long-term complications were identified in the thoracoscopic cohort. The median follow-up was 27 months. CONCLUSIONS: In our experience, thoracoscopic CDH repair was performed safely and with similar outcomes compared to open repair. In addition to improved cosmesis, thoracoscopic repair may avoid some of the long-term complications of laparotomy. In our series, none of the thoracoscopic CDH repairs recurred. We conclude that thoracoscopic CDH repair is a safe and appropriate technique for select neonates.

Predictors and Outcomes of Nondiagnostic Ultrasound for Acute Appendicitis in Children.

Ultrasound assessments of children with possible acute appendicitis (AA) are often nondiagnostic. We aimed to identify the predictors of nondiagnostic ultrasound and to investigate the outcomes. A retrospective review was conducted on children aged 4 to 17 years evaluated in 2013 for AA with ultrasound at a tertiary hospital pediatric emergency department. Demographics, clinical data, and outcomes were analyzed. Of 528 children, 194 (36.7%) had diagnostic ultrasounds and 334 (63.3%) had nondiagnostic ultrasounds. Nondiagnostic ultrasounds were more common after-hours (7 pm-7 am weekdays and on weekends, 70.7%) than during business hours (7 am-7 pm weekdays; 29.3%). After-hours timing and female sex were identified as independent predictors of nondiagnostic ultrasounds (P < 0.05 for both). AA was diagnosed in 35 children with a nondiagnostic ultrasound (10.5%; P < 0.05). No child who underwent a nondiagnostic ultrasound was found to have AA with laboratory values of white blood cell < 11 × 103/µL and c-reactive protein (CRP) < 5 mg/dL. Children with nondiagnostic ultrasounds have a low likelihood of AA if white blood cell < 11 and CRP < 5. We propose a management algorithm that we hope will help reduce admissions and decrease the use of computed tomography scans.

A near lethal case of plastic bronchitis following truncal valve replacement.

Plastic bronchitis is a condition in which large, rubber-like, bronchial casts develop in the tracheobronchial tree causing airway obstruction. It is an unusual disorder that occurs in various disease states. Most case reports of plastic bronchitis associated with congenital heart disease are in patients palliated with Fontan physiology. We describe a 13-year-old girl with DiGeorge syndrome and truncus arteriosus who underwent an uneventful truncal valve replacement. The child developed plastic bronchitis on postoperative day eight requiring extra-corporeal membrane oxygenation support and daily bronchoscopy to remove bronchial casts. Our patient did not have elevated systemic venous pressures or Fontan physiology. We speculate the etiology of plastic bronchitis may have been surgical trauma to the lymphatic channels surrounding the bronchi and disruption of pleural adhesions. A comprehensive evaluation revealed no specific cause and the diagnosis of idiopathic plastic bronchitis was made.

Return of the surgeon in the diagnosis of pyloric stenosis.

BACKGROUND: The diagnosis of pyloric stenosis (PS) by physical examination is a lost art that has been replaced by radiology-performed ultrasound (US). The purpose of this study is to demonstrate that the diagnosis of PS can be made solely upon the surgeons US evaluation. METHODS: Surgical ultrasonographers included 2 senior general surgery residents and 2 pediatric surgery residents without prior formal US experience. These surgeons underwent proctored training in the use of US for PS. Measurements including channel length and muscle thickness were recorded at bedside. A positive examination included muscle thickness more than 4 mm and channel length more than 16 mm. Patients with positive results underwent pyloromyotomy. Negative results were confirmed with a repeat US through the radiology department, and infants without PS were subsequently referred for appropriate medical management. RESULTS: Thirty-two consecutive patients with suspected PS were evaluated using surgeon-performed ultrasonography. All examinations were diagnostically accurate. There were no false-positive or false-negative result. Seven patients (22%) were correctly determined to be negative for PS. The remaining 25 infants underwent successful pyloromyotomy with resolution of symptoms. CONCLUSION: Surgeons who have undergone focused training to perform US for PS can diagnose the condition without confirmatory testing by a radiologist.

Tracheobronchial injury diagnosed by computed tomography scan: a case report.

Tracheobronchial injuries are uncommon after pediatric blunt trauma. Because they are easily missed and potentially life-threatening, surgeons caring for pediatric patients with trauma should be aware of this spectrum of injuries. We present a case of tracheobronchial injury in a 10-year-old girl caused by blunt trauma sustained during an all-terrain vehicle collision. This injury was diagnosed in a timely fashion with computed tomography scan, and operative repair was accomplished. A discussion of the management of tracheobronchial injury with brief literature review follows.

Surgeon-performed ultrasound for diagnosis of pyloric stenosis is accurate, reproducible, and clinically valuable.

PURPOSE: We will demonstrate that a surgical resident with proven accuracy in the diagnosis of hypertrophic pyloric stenosis (HPS) can teach other surgeons to diagnose HPS with reproducible accuracy. METHODS: A surgical resident with proven sonographic accuracy in diagnosing HPS instructed 5 other surgical residents in the technique. Consecutive patients referred to pediatric surgery with a presumed clinical diagnosis of HPS were examined, and measurements of residents were compared with formal radiology studies. Each surgeon was proctored for 5 examinations before independent evaluation and was blinded to results from both radiologists and other residents. Results were evaluated using Student's t test; P less than .05 was considered significant. RESULTS: Seventy-one patients were evaluated by 5 surgical residents. Residents were diagnostically accurate in all cases. There was no statistically significant difference between pyloric muscle thickness or channel length measurements obtained by radiology and any of the residents. CONCLUSION: Surgeon-performed ultrasound examination for the diagnosis of HPS is accurate and reproducible through surgeon-to-surgeon instruction on appropriate technique. This skill is a valuable asset in the initial surgical evaluation of any patient with suspected HPS, expediting appropriate management.

Residency training in pyloromyotomy: a survey of 331 pediatric surgeons.

PURPOSE: Both pediatric and general surgeons perform pyloromyotomy. Laparoscopic pyloromyotomy (LAP), and changes in referral patterns have affected the training of pediatric surgery fellows and general surgery residents. We surveyed pediatric surgeons regarding these issues. METHODS: We mailed an Institutional Review Board of New Hanover Regional Medical Center-approved survey to 701 members of the American Pediatric Surgical Association within the United States to determine each surgeon's preferred technique for pyloromyotomy (LAP vs Ramstedt or transumbilical procedures [OPEN]), practice setting, involvement with trainees, and opinions regarding pyloromyotomy. Significance was determined using chi(2) analyses. RESULTS: A total of 331 (48%) surgeons responded: 197 (60%) performed most or all OPEN, and 85 (26%), most or all LAP. Laparoscopic pyloromyotomy was more likely in academic practices and children's hospitals (P < .05). Residents under surgeons performing LAP were less likely to participate (58% vs 91%; P < .05) or gain competence (22% vs 42%; P < .5). Only 34% of surgeons performing LAP believed that general surgery residents should learn pyloromyotomy, whereas 67% of surgeons performing OPEN believed that residents should learn the procedure (P < .05). A total of 307 (93%) surgeons believed at least 4 OPEN were necessary to become competent, but 126 (44%) reported that their residents performed fewer than 4. Only 104 (31%) surgeons believed that their residents were competent in pyloromyotomy. There were 303 (92%) surgeons who believed that pyloromyotomy should be performed only by pediatric surgeons when possible. CONCLUSIONS: Most general surgical residents are not learning pyloromyotomy, in part because of the adoption of laparoscopic technique, limited operative experience, and the opinion of most pediatric surgeons that the procedure should be performed only by pediatric surgeons.

Hospital characteristics affect outcomes for common pediatric surgical conditions.

Appendicitis, hypertrophic pyloric stenosis (HPS), and intussusception are common conditions treated in most hospitals. In which hospital settings are children with these conditions treated? Are there differences in outcomes based on hospital characteristics? Our purpose was to use a nationwide database to address these questions. Data were extracted from Kids' Inpatient Database 2000. Data were queried by International Classification of Diseases procedure code for appendectomy and pyloromyotomy and by diagnosis code for intussusception. Length of stay (LOS) and hospital charges were analyzed based on hospital size, location, teaching status, and specialty designation. There were 73,618 appendectomies, with 5,910 (8%) in children's hospitals. Overall LOS was 3.1 days, and was the longest in children's hospitals (3.9). Overall charges were dollar 10,562, with the highest in children's hospitals (dollar 14,124). There were 11,070 pyloromyotomies, with 2,960 (27%) in children's hospitals. Overall LOS was 2.7 days, the shortest being in children's hospitals (2.5). Overall charges were dollar 7,938, with the highest in children's hospitals (dollar 8,676). There were 2,677 intussusceptions, with 921 (34%) in children's hospitals. Overall LOS was 3.0 days, the shortest being in children's hospitals (2.8). Overall charges were dollar 9,558, with the highest in children's hospitals (dollar 10,844). Most children with appendicitis, HPS, and intussusception are treated in nonspecialty hospitals. HPS (27%) and intussusception (34%) are more likely than appendicitis (8%) to be treated in children's hospitals. Children's hospitals have higher charges for all three conditions despite shorter LOS for HPS and intussusception.